b -hexosaminidase A
Please Answer the Following Questions (3 paragraphs). Remember–do not to include your full name in your posts (example: John T. Period 3).
http://www.marchofdimes.com/pnhec/4439_1227.asp
(useful link but you made need to access other resources)
1. What is Tay-Sachs disease and who does it affect?
2. What are the symptoms of Tay-Sachs? What is the prognosis for a child with Tay-Sachs disease?
3. Explain how enzymes play a role in the genetic disorder. Be detailed.
Alex Sp wrote @
1.) Tay-Sachs disease is fatal and orginates in the central nervous system, and affect babies. It later makes the baby lose simple skills, and form much more serious problems such as paralyzation.
2.) The symptoms of tay-sachs disease include stopping smiling, crawling, turning over and reaching out. Later, the baby loses skills gradually and can eventually become blind, paralyzed and is unaware of surroundings. These sorts of things happen because there is a lack an enzyme (protein) called hexosaminidase.
3.) Enzymes play a crucial role because of the two types of the enzyme, hexosaminidase, hex A and hex B, the baby with tay-sachs disease only has hex B. The enzyme, hexosaminidase is necessary for breaking down certain fatty substances (called GM2 gangliosides) in cells of the brain.
Jessie B. wrote @
Tay-Sachs disease is a very dangerous disease. It is an inherited disease of the central nervous system. Tay-Sachs disease mainly affects babies. It is caused by a mutation in a gene.
Tay-Sachs disease has many symptoms. These symptoms begin when the baby is about six months old. It begins when a baby stops smiling, crawling, turning over, and reaching out. The baby will keep losing various skills until he becomes paralyzed, blind, and unaware of his surroundings. A child with Tay-Sachs disease will most likely die before they are five.
Babies who have Tay-Sachs disease don’t have an enzyme called hexosaminidase. Hexosaminidase is an enzyme which breaks down fatty acids in the brain. Without it, the fatty acids pile up and desroy brain cells until the nervous system stops working. Tay-Sachs disease is a dangerous disease with horrible symptoms.
Alex R wrote @
1) Tay-Sachs disease is an inherited disease that’s affects the central nervous system. They are caused by mutations in different genes. A form of each disease affects babies and is fatal.
2) The symptoms of Tay-Sachs generally appear about when they are 6 months old when a baby who looks healthy gradually stops smiling, crawling, turning over, and reaching out. The baby continues to lose its skills and will become blind, paralyzed, and unaware of its surroundings. The baby will usually die by age 5.
3) Enzymes play a roll in genetic disorders because enzymes have catalyst which speeds up reactions and when the enzymes are missing, the body can’t produce these functions and the body will slow down. The metabolism will slow down and the body will not be able to survive and all the skills that we have will be lost.
Bradford (Ford) F. wrote @
1. Tay-Sachs Disease is a hereditary disease that causes gradual loss of bodily functions at a certain age. There are three possible age groups who may be affected: six-month old babies, children ages 2-10, and mid-life s. Tay-Sachs patients must have two parents who are genetic carriers; those people who do have two parents who are genetic carriers therefore have a 25% chance of having the Tay-Sachs gene passed on to them. The disease is mostly known to run through Jewish heritage.
2. The typical symptoms of the Tay-Sachs Disease vary depending on age. Babies with the disease will first lose their ability to hear, see, and touch. Then, they will begin to lose mobility until they are completely paralyzed. Eventually, they will die usually by age five.
The typical juvenile symptoms for children 2-10 are basically the same only milder and occurs around age 15.
Symptoms for s often begin around age 30 and are far less extreme. Loss of vision or hearing does not occur. Instead, they will experience memory loss and loss of some common mental abilities. Age at varies, and often the person with the disease as an will not die from it.
3. Tay-Sachs Disease is simply the result of a gene that makes the person who has it lack the enzyme hexosaminidase. Hexosaminidase is necessary for breaking down some fatty substances in cells of the brain. Without this enzyme, these fatty substances build up and gradually destroy brain cells, until the entire central nervous system stops working. When this buildup begins, the first symptoms begin to show.
Eleanor H period6 wrote @
1. Tay- Sachs disease is a neurological genetic disorder. The disease is caused by the absence of a vital enzyme called Hexosamindase A. Tay-Sachs affects babies, because they are born with the disease. There is an increase in the disease in Eastern European Jewish (Ashkenazi), though Tay-Sachs can affect everyone. The disease causes destruction of the central nervous system because they are missing the enzyme that breaks down gangliosides.
2. Symptoms of Tay-Sachs are found among babies and include different things. One symptom of Tay-Sachs is that development is slow, and eventually stops. Another symptom of Tay-Sachs is the loss of peripheral vision. Seizures are also another symptom though they do not always occur. Eventually someone who is effected with the disease will become blind, deaf, paralyzed, mentally retarded, and completely unable to interact with or respond to the environment they are in. When a child has Tay-Sachs, they have a red spot in the back of their eyes. The prognosis is not completely certain because this is a newly discovered disease, but the prognosis now is death. There are no treatments for Tay-Sachs so the prognosis of death usually occurs around age 5.
3. Enzymes play a huge role in the genetic disorder Tay-Sachs. The reason people get the disease Tay-Sachs is because they are missing a critical enzyme that breaks down the fatty substance Hexosamindase A. This enzyme is found in lysosomes which are cellular organelles; It is located on chromosome 15. Because the enzyme is not present it causes a build up of ganglioside in neurons. Due to the neurons location (outside the nervous system) this build up slowly causes the nervous system to slowly break down.
Shannon G. wrote @
1) Tay-Sachs is a disease found in the central nervous system. It can be inherited genetically, because of mutations in genes. Because it can be inherited, it affects babies, specifically descendents of Central and Eastern European Jews.
2) Symptoms of Tay-Sachs disease include the gradual decrease of smiling, crawling, turning over, and reaching out of a baby’s behavior. The baby will continue to gradually lose skills, and could become blind, paralyzed, and unable to identify with their surroundings in the future. The prognosis of a child with Tay-Sachs is death before the age of 5.
3) Enzymes play a role in this genetic disorder. Babies with Tay-Sachs lack hexosaminidase, an enzyme. As a result, GM2 gangliosides, a fatty substance found in cells of the brain cannot be broken down. This causes the substances to build up. This will destroy brain cells gradually; eventually the whole central nervous system will no longer function.
Katie H wrote @
1Tay-Sachs is an inherited disease that affects the central nervous system. This disease is caused by mutations in different genes. Tay-Sachs affects babies and can kill them.
2 The symptoms of Tay-Sachs are that they appear healthy at birth and begin to develop normally for the first few months. At about 6 months the baby stops smiling, crawling, turning over and reaching out. The baby keeps lossing simple skills and eventually becomes blind, paralyzed and unaware of its surroundings. They usually die by the age of 5.
3 Enzyames play an important role in Tay-Sachs. Babies with this disease lack an enzyme called hexosaminidase. This enzyme has two versions hex A and hex B and babies with the disease make both of them but lack the protein that is needed to make these enzymes work right. This enzyme is so important because it is used to break down fatty substances in the cells of the brain. Without the enzyme the fatty acids build up and distroy the brain cells untill the brain stops working.
Martin C period 6 wrote @
1. Tay sachs disease allows gangliosides, a fatty substance on the cells in the brain, to build up to deadly amounts. Tay sachs disease mainly affects children under the age of five. Babies often develop symptoms at six months old. But there is another form of the disease that affects people as old as 30,
2. The first symptoms occur around 6 months old and include stopping smiling, crawling, turning over and reaching out. As the baby gets older it begins to lose skills gradually and eventually becomes blind, paralyzed and unaware of surroundings. this unfortunately becomes fatal by the age of 5.
3.Enzymes play an important role in tay sachs disease because the enzyme hexosaminidase is no longer produced. When this enzyme is not produced it allows a fatty substance to build up in the brain. There are two kind of hexosaminidase hexA and hexB. Babies without the enzyme hexA have tay sachs disease.
sarah lev wrote @
1.Tay-Sachs disease is an inherited disease of the centeral nervous system and is cause by mutations or changes in genes. this disease affects children and babies.
2.Babies with this disease appear healthy at birth and develop normally for the first few months. Symptoms start to appear by about 6 months. some symptoms are when the child stops smiling, crawling, turning over or reaching out. The baby keeps losing skills untill they become blind, paralyzed and unaware of thier surroundings. Babies with Tay-Sachs disease usually die by age 5.
3. Babies with this disease dont produce an enzyme called Hex A. this Hexosaminidase is necessary for breaking down fatty substances in cells of the brain. Without this enzyme, these substances build up and destroy brain cells, until the central nervous system stops working. some babies produce Hex A but are lacking a protein that is necassary for the enzyme to work properly.
Jeff Q wrote @
1. Tay-Sachs disease is a central nervous system disease that affects infants through inheritance and can cause death in some of the infants that it is found in.
2. The symptoms of Tay-Sachs usually start to occur at around six months of age. At this time they start to lose skills such as smiling, crawling, rolling over and reaching out. They also become blind and unaware of their surroundings. People usually die from Tay-Sachs around the age of five.
3. Babies with Tay-Sachs lack the enzyme hexosaminidase. There are two types of this enzyme, hex a and hex b. Babies with Tay-Sachs don’t make hex a which breaks down a fatty substance in the brain that will eventually destroy it if not brocken down by this enzyme.
Brian wrote @
1) Tay-Sachs disease is a disease of the central nervous system that primarily affects infants. The disease occurs when the infant doesn’t get enough enzymes
2) You don’t notice any symptoms until the baby is at least 6 months old. Some of the symptoms are, the baby gradually stops smiling, crawling, turning over and reaching out. The infant continues to lose more skills, and becomes blind, paralyzed, and unaware of its surroundings. The baby eventually dies by the age of 3, if not earlier.
3)the enzyme hexosaminidase, is necessary for breaking down certain fatty substances in cells of the brain. Without this enzyme, these fatty substances build up and gradually destroy brain cells, until the entire central nervous system stops working.
Jake G. Period 4 wrote @
1.Tay- Sachs disease is a disease that affects mainly toddlers. It is a genetic disease that happens when a baby lacks and enzyme. THis causes the brain to be filled with fat which kills brain cells.
2.The symptoms occur usually around 6 months into a child’s life. The toddler will learn to crawl and laugh before the disease kicks in but then the baby will began to lose all the skills it has learned. The child will most likely die and if symtoms happen early the baby will probably die by the age of 5.
3.Enzymes are important to the disease. The enzyme is called hexosaminidase this enzyme breaks down fatty substances in brain cells. Not having this enzyme causes the disease because without it the fatty substance kills the brain cell.
Abby W. wrote @
Tay-Sachs disease is a fatal disease that affects the developing nervous systems and brains of young babies. Tay-Sachs disease is a genetic, in which the baby is born without a vital enzyme called Hexosaminidase A or Hex A. This enzyme clears out unwanted fatty protein and other materials that are interfering with the development of the baby and its basic functions. As fatty protein and waste build up in the baby’s brain, it inhibits the normal neurological development of the child.
This tragic disease can at first be undetected because for the first few months of the child’s life it seems to develop normally, but gradually it begins to show symptoms and loose its ability to function normally. The disease is usually diagnosed in the baby’s first 3-6 months of life if the baby is having hearing, sight or movement problems. As the disease progresses the baby typically shows more signs of the disease like total loss of sight, by not smiling anymore, and becoming less active. If the baby reaches age 2 it will most likely become paralyzed by that time or have severe seizures. The grim prognosis for babies with Tay-Sachs disease is death by age 5. There are some forms of the disease in which develops more slowly, for example the juvenile Tay-Sachs disease and symptoms begin around age 5 but death is still expected by age 15.
Enzymes play a huge role in this genetic disorder. The enzyme beta-Hexosaminidase A clears fatty substances called ganglioside from the cells and nervous tissue in babies brains. Infants with Tay-Sachs disease lack this enzyme and therefore have buildup in their brains of this material. The fatty protein builds up in the cells and causes the neurological functioning problems in babies that is called Tay-Sachs disease. This is one example of how important enzymes are to the proper functioning of our bodies, and exemplifies the disastrous consequences of not containing these crucial catalysts.
Jacqueline S. Period 6 wrote @
1. Tay-Sachs disease is an inherited disease of the central nervous system. It is caused by mutations (changes) in different genes. This disease affects babies and is fatal.
2. The symptoms of Tay-Sachs are that the baby appears to be healthy at birth and seems to develop normally for the first few months of life. Then symptoms appear by about 6 months of age when an apparently healthy baby gradually stops smiling, crawling, turning over and reaching out. The baby continues to lose skills gradually and eventually becomes blind, paralyzed and unaware of surroundings. Babies with Tay-Sachs disease usually die by age 5.
3. Enzymes play a role in the genetic disorder by the babies with Tay-Sachs disease lack an enzyme (protein) called hexosaminidase. There are two types of this enzyme, hex A and hex B. Babies with Tay-Sachs disease do not make hex A. A small number of babies with Tay-Sachs disease (AB variant) make both types of the enzyme, but lack another protein that is needed for these enzymes to work properly. Hexosaminidase is necessary for breaking down certain fatty substances (called GM2 gangliosides) in cells of the brain. Without this enzyme, these fatty substances build up and gradually destroy brain cells, until the entire central nervous system stops working.
Brianna I. P4 wrote @
1. Tay-Sachs is an inherited disease of the central nervous system. It is caused by mutations in different genes. It affects babies and is found primarily (but not only) among Ashkenazi Jews – those of East European ancestry.
2. Symptoms of Tay-Sachs disease include appearing healthy at their birth and the first few months of their lives. At around 6 months old babies that appear healthy gradually stop smiling, crawling, turning over and reaching out. The baby continues to lose skills gradually and ultimately become blind, paralyzed and unaware of surroundings. A child that has Tay-Sachs will die around the age of 5 because there is not treatment for the disease.
3. Enzymes that are not present or do not function correctly play a role in genetic disorders. Tay-Sachs is caused by the absence of a gene called hex A. The absence of the gene results in absence of the metabolic pathway necessary to prevent the deposition of damaging fatty deposits in nerve cells. Carriers of the gene make about 50% less of the enzyme required which causes Tay-Sachs.
Michael S. wrote @
1:Tay -Sachs disease is a disease that infects the central nervous system, causing a child to grow up differently, making it blind and have other terrible sideaffects. it sooner or later leads to death. The disease effects baby’s who carry this disease, and also could be effected later in life.
2:Symptoms include, bilndness, paralyziation, unaware of its surrondings, and loss of skills.
3: The carriers of Tay- Sachs are lacking the Enzyme Hex A and babies with sandhoff don’t have enzyme A or B
Derek period 4 wrote @
1. Tay-Sachs disease is a disease that is inherited from your parents and previous ancestors. The disease infects the enteral nervous system. People who are diagnosed with the disease are babies.
2. You can tell when a baby has tay-sachs when they stop crawling, smiling, or never want to turn over. Eventually the baby will go deaf and become unaware of its surroundings. From then on its all down hill. The worst part of it all is that there is no cure for the disease meaning the only thing they can do is make the baby as comfortable as they can and wait for it to pass on.
3. Enzymes are the main reason in which he disease exists. The disease is because a certain enzyme is not present. Without this enzyme the body can not break down fats and they will build up in the brain. This will disrupt the nervous system resulting in eventual death. But if the enzymes where present the disease would cease to exist.
Nicole C. wrote @
1. Tay Sachs a disease caused by the absence of the enzymes hexosaminidase A and B from in the body. The inability to break down certain substrates causes an unhealthy build up of fatty substances in the brain which essentially kills off the brain cells until death. Victims of Classic Tay Sachs are almost always babies and toddlers because by age five, the child’s brain has basically rotted away. The central nervous system stops functioning and death is not far behind. In late-onset Tay Sachs, the person can survive without symptoms for 30 years. Descendents of Ashkenazi Jews and French-Canadians are at increased risk for being born with this disease.
2. Symptoms of Tay Sachs disease present themselves during infancy. The baby gradually stops developing and begins slipping backward until it loses sight, ability to move (paralysis), and then they don’t even know or care what goes on anymore. Within five years, the child will be dead. In late-onset Tay Sachs, symptoms include slurred speech, memory loss, mental illness, tremors, muscle weakness, and problems with comprehension. Death is not always imminent in mild cases of late-onset but in most cases, this disease is fatal.
3. The main cause for the disorder itself is the lack of an enzyme, hexosaminidase. Every enzyme has the job of speeding up the chemical reactions necessary to break bonds. Without these enzymes, it would be difficult, if not impossible for the reactions to take place on their own. Tay Sachs disease is an example of what it would be to lack even one enzyme. In this case, the results are catastrophic. Because there is no enzyme there to break down the substrate, it cannot be used, changed, or absorbed by the body. Therefore, it just keeps building up. As it builds up, it destroys the brain and central nervous system which will eventually lead to death. All of these symptoms occur because the person had the genetic disorder which left them without one enzyme.
Miranda D. Peroid 6 wrote @
1. Tay-Sachs is a disease of the central nervous system caused by a mutation in chromosome 15. It generally affects those of eastern and central European Jewish heritage. However, it also affects people from the Lawrence river valley in Quebec and the Cajun population in Louisiana.
2. The symptoms of classic Tay-Sachs disease appear after about six months of age. The child will stop smiling, turning over, reaching out, and crawling. Then the child will then continue to lose skills until it becomes blind, deaf, and unable to swallow. Children with this disease will probably die by age five, as there is no treatment or cure.
3. Tay-Sachs disease is characterized by the absence of hexosaminidase A, an enzyme that breaks down fatty tissue in the brain. On occasion the child will have the enzyme but lack the protein needed by the enzymes (substrate). Without this enzyme the fatty tissue will build up and eventually kill brain cells. This will lead to the failure of the entire nervous system.
kailey-p4 wrote @
1) Tay-Sachs disease affects the central nervous system and are caused by mutations in different genes. The disease mainly acts up on babies.
2) The symptoms for Tay-Sachs starts when the baby has reaches six months old, untill then the baby ususually would have seemed to develop normally. The baby slowly starts to lose skills such as smiling, crawling, turning over and reaching out. Eventually the baby will become blind, paralyzed and completly unaware of surroundings. The average life expectancy for babies living with the disease is 5 years, and there is no known treatment for the disease.
3) Heritable genetic disorders occur because there is a deficiency of one or more enzymes, and they are unable to break down the substrate correctly .
Bridget P. ~3 wrote @
Tay- Sachs disease affects the central nervous system. It is most commonly found in relatives of Central and Eastern European (Ashkenazi) Jews. This disease is inherited from the parents. In America, about 1/30 of all Jews carry the mutated gene that causes this fatal disease. Most commonly, Tay-Sachs affects children from 6 months to 5 years. Another form is called Juvenile Tay-Sachs. It begins to develop between 2 and 10 years old. Adult-onset tay- sachs is a milder condition where symptoms begin from adolescence until 30s.
Symptoms of Tay-sachs in infants are apparent when they stop crawling, stop reaching out, stop smiling, and/or stop turning over. They gradually become less active and may become blind, paralyzed, and unaware of what is happening around them. An infant with Tay-sachs will most likely die before age 5. An older child with juvenile tay-sachs will die by age 15. Adult-onset tay-sachs disease will not be as severe, for it is a milder case and can be dealt with more easily. Symptoms of this include short term memory loss and slurred speech. Typically adults do not loose their hearing of sight.
Individuals affected by Tay-sachs disease lack hexosaminidase. This enzyme is crucial to the breaking down the brains specific fatty substances. Without the enzyme, hexosaminidase, brain cells are destroyed by the build up of fatty substances. Eventually the central nervous system will cease to function entirely.
Alex D. Period 4 wrote @
1.Tay-Sachs is a disease which attacks the central nervous system in the body. The disease is caused by mutations in differing genes. Infants are most often affected, and it is also found more commonly in certain backrounds in comparison to others. Eastern and Central European Jews, as well as French Canadians have a higher tendancy to carry and receive the gene. However, if someones parents carry the mutation, then that person stands a 25% chance of inheriting the gene itself.
2.Tay-Sachs strikes hard and fast, but infants with the disease will lead active and healthy lives for the first few months of their existance. However, around 6 months time, the symptoms of the disease begin to show their ugly faces. A baby will gradually begin to cease smiling, reaching out, turning over or even crawling. Even more basic skills continue to be lost, and the result is loss of sight, paralysis, and becoming unaware of ones surroundings. Infants who inherit Tay-Sachs disease often die around the age of 5, when their central nervous system just shuts down completely. If the disease is found in an infant, it is like a death sentence, because there is no known cure for Tay-Sachs, and the baby will degress until it dies.
3.Enzymes are crucial when it comes to Tay-Sachs disease, because it is caused by the lack of an certain enzyme. Hexosaminidase is an enzyme which breaks down fatty substances in the brain, and is found in every healthy human. However, people with Tay-Sachs are lacking a type of hexosaminidase, called hex A, and without it, the fatty substances begin to build up and destroy brain cells. This occurence wrecks apart the central nervous system, causing a very quick death. If this enzyme was present in every human, there would be almost no cases of Tay-Sachs disease.
Mary C wrote @
1.) Tay-Sachs disease is inherited diseasesof the central nervous system. It affects babies.
2.)They symptoms for Tay-Sachs disease don’t become noticeable til about 6 months and then the baby loses its skills gradually and eventually becomes blind, paralyzed and unaware of surroundings.Babies with Tay-Sachs disease usually die by age 5.
3.) Babies withTay-Sachs diseases lack an enzyme called hexosaminidase. Babies with Tay-Sachs disease do not make hex A. Hexosaminidase is necessary for breaking down certain fatty substances in cells of the brain. Without this enzyme, these fatty substances build up and gradually destroy brain cells, until the entire central nervous system stops working.
Shannon B wrote @
1.) Tay-Sachs disease is an inherited disease of the central nervous system. This disease effects babies and is fatal. The disease is passed on through parents who carry it.
2.) Some symptoms for Tay-Sachs symptoms generally appear by about 6 months of age when an apparently healthy baby gradually stops smiling, crawling, turning over and reaching out. The baby continues to lose skills gradually and eventually becomes blind, paralyzed and unaware of surroundings. Babies with Tay-Sachs disease usually die by age five, and those with Sandhoff disease by age three. There is currently no treatment that will prevent these diseases from running their course. There is no prognosis that will prevent this disease from running its course. Affected children can only be made as comfortable as possible and given other supportive care.
3.) Babies with classic Tay-Sachs and Sandhoff diseases lack an enzyme called hexosaminidase. There are two versions of this enzyme, hex A and hex B. Babies with Tay-Sachs disease do not make hex A and some babies with Tay-Sachs disease make the enzyme, but lack another protein that is needed for these enzymes to work properly. Hexosaminidase is necessary for breaking down certain fatty substances in cells of the brain. Without this enzyme, these fatty substances build up and gradually destroy brain cells, until the entire central nervous system stops working. This is when a baby is diagnosed with Tay-Sachs disease.
Steven J. Period 4 wrote @
1. Tay-Sachs disease is an inherited disease of the central nervous system. This disease results from mutations in different genes. This disease normally affects babies and has proven to be fatal.
2. Symptoms of Tay-Sachs disease generally do not appear until the child is about six months of age. At this point the apparently healthy baby gradually stops smiling, crawling and turning over. This progresses until the baby becomes blind, paralyzed, and unaware of its surroundings. Babies with the disease usually die by the age of five.
3. Babies with Tay-Sachs lack the enzyme hexosaminidase. This enzyme is necessary for breaking down fatty substances called GM2 gangliosides in cells of the brain. Lacking this enzyme forces these to build up and progressively destroy brain cells until the point when the whole central nervous system seizes to functioning.
Justin S p4 wrote @
1. Tay Sachs disease is an inherited disease of the central nervous system. Babies usally have this disease when they are born. They grow normally until age 6 months then the disease takes over because they lack the enzyme hexosaminidase A. Without this enzyme the fatty acids slowly destroy the brain cells.
2. The symptoms are this disease are dangerous. The symptoms start slowly from being unable to crawl until the baby loses all skills, becomes blind, and then is paralized and unaware of its surroundings. The baby will die by age 5.
3. Enzymes play a major role in this disorder. The lack of the enzymes hexosaminidase A and B causes this disorder. Without these enzymes the fatty acids in the brain slowly destroy the cells until the baby’s central nervous system shuts down.
Chris wrote @
1. Tay-Sachs is a disease you recieve when you are an infant and it is a disorder you get from not having hexosaminidase.
2. Tay-Sachs is a disease that takes away skills such as sight and gait. At age 5 the infant dies. The prognosis for this is death but you can amke them comfortable.
3. This is caused by a missing enzyme called hexosaminidase. This breaks fatty acids in your brain and without them they affect your brains
Danny B – Period 3 wrote @
Tay-Sachs disease is a genetic mutation that occurs most commonly in the infant stages of life, but is not exclusive just to this age range. It is an inherited disease that slowly destroys the central nervous system due to the lack of an enzyme called hexosaminidase.
Tay-Sachs disease has many symptoms that affect a carrier of the genetic mutation. As the disease starts to degrade the cells in the brain of a carrier, usually six months after birth in infantile cases, the carrier will gradually stop smiling, turning over, and performing other activities most young children would do. As the diseases worsens, the carrier will lose more motor skills gradually and ultimately become paralyzed and blind and have totally lost all awareness of his/her surroundings. The prognosis for Tay-Sachs disease, since there is no treatment, is that the carrier will eventually die as the disease further worsens and degrades the central nervous system.
Enzymes play a vital role in the cause of most classic cases of Tay-Sachs disease. The enzyme, as mention before, is called hexosaminidase and it comes in two types in the human body, Hex A and Hex B. When a baby does not produce Hex A naturally in it’s body, then the baby is considered to have Tay-Sachs disease. However, Tay-Sachs disease can occur when a carrier makes both Hex A and Hex B, but is missing an alternative protein, which assists them in functioning properly. When this enzyme is not produced in a child, it prevents the body from breaking down certain fatty acids called GM2 gangliosides that are stored in brain cells. Over time, these fatty acids build up and gradually destroy brain cells in a carrier of Tay-Sachs.
chris lep wrote @
1. Tay-Sachs disease is a disease that affects mostly Jews and some non-Jewish french Canadians.
2. The symptoms of Tay-Sachs disease is if a baby just stops smiling after a while and eventually doesn’t do anything at all. The prognosis of a child with Tay-Sachs disease is death.
3. Enzymes play a role in genetic disorder because if you are missing an enzyme then you can have serious diseases like this one.
Brittany J wrote @
1. Tay-Sachs disease is an inherited disease caused by a mutation in certain genes and they lack an enzyme called hexosaminidase. Tay-Sachs doesn’t produce the version hex A and Sandoff doesn’t make the hex B version. This diesease is fatal and mostly affects babies.
2. There are many symptoms of Tay-Sachs. The changes are gradual, but the baby eventually stops smiling, crawling, turning over, and reaching out. The baby then gradually loses important skills and becomes blind, paralyzed, and unaware of what is going on around them.
3. Enzymes play an important role in the genetic disorder. Without the enzyme hexosaminidase, the healthy baby becomes more and more unable to function properly just becuase of one absent enzyme. Although the chances are slim, not having that enzyme changes a person’s ebtire life, cutting down their time to live dramatically.
Doug R. wrote @
1. Tay-sachs affects babies and it is a inherited disease in the central nervous system.
2. The baby stops smiling, crawling, and reaching out. Then gradually it becomes unaware of its surroundings and is completely paralyzed and blind. The prognosis is death.
3. The enzymes related to this disorder are called hexosaminidase A and B. Babies with Tay-sachs are not born with hexosaminidase A and therefore cannot break up the fatty acids that buildup in the brain. The enzyme brings the fatty acid together and breaks its bonds and without the enzyme it would be impossible to break them.
mattb period 3 wrote @
1 It is a metabolic disorder that affects Ashkenzai jews and french canadians and cajuns of L.A.
2 dementia and blindness and early death a baby will stop crawling smiling and not be aware of its surroundings.there is no treatment to stop it from killing some one they should be givin comfort
3 to get this diesase a baby lacks an enzyme called hex a and hex b in their body and stops development and dies. its like it stops its growing all togeter and goes backwards untill he or she dies
Alan S P6 wrote @
1) Tay-Sachs disease is a disease that newborn babies inhereit beacuse they don’t have a specific enzyme. This obviously affects these unlucky babies, and death is the evetual outcome for these babies, usually at the age of about five.
2) If a baby has Tay-Sachs disease, they usually develop normally for the first few months, until the age of about 6 months. At this time the babies gradually stop smiling, crawling, and turning over and reaching out. The symptoms then continue to get worse as the baby’s skills worsen and they eventually become blind and paralyzed. Death normally occurs at about the age of five for these unlucky individuals.
3) In general, most disorders like Tay-Sachs are caused because the brain stops working for one reason or another. In this specific case, the babies who are victim to the disease lack an enzyme called hexosaminidase. This enzyme breaks down fatty substances that are present in the brain. When this enzyme doesn’t work, these fatty substances pile up, eventually causing the symptoms noted above. Basically, the lack of the needed enzymes is what causes this unfortunate disease.
MayaZ p3 wrote @
1) Tay – Sachs disease is a fatal genetic disorder that affects the nervous system. Babies with Tay -Sachs disease live to be about five years old. These children do not make hexosaminidase A, which is needed to break down certain substances. They have a genetic mutation on chromosome 15 and therefore do not produce said enzyme. Although there is no cure for this disease, stem cell transplants and certain drugs have the potential to stop or reverse Tay – Sachs yet there has not been any proven success.
2) A child with Tay – Sachs will present symptoms around six months. The child’s development will reverse until they are no longer smiling, crawling etc. Eventually the child will become blind and paralyzed. There is no cure, although researchers are working on different possible treatments. Doctors are able to keep the child comfortable. There are tests for a person to find out if they are a carrier for this disease, as well as a test to find out whether an unborn child will have Tay – Sachs.
3) Tay – Sachs is a genetic disease that occurs when a child doesn’t produce the enzyme hexosaminidase A. This protein breaks down fatty substances in the brain. Without it, the fatty substances will build up in the brain and destroy cells. This explains why a child with this disease will progressively lose certain abilities. The drug miglustat could potentially reduce the build-up for fatty substances but its effectiveness has yet to be proven.
Stephen B wrote @
1. Tay- Sachs is a disease that you inherit from your parents. This disease effects the central nervous system.
2. Symptons of Tay- Sachs are the baby stops smiling, crawling, turning over and reaching out. This usaully occurs after 6 months of the baby being alive. The childs skills eventually worsen until he/she becomes blind and paralyzed. Then they die by the age of 5.
3. Enzymes plays a major role in genetic disorders. A great example is Tay- Sachs disease which the person lacks the enzyme hexosaminidase. This means people with this disease do not produce hex A, which is a protein that breaks down certain fatty substances in the brain that gradually destroys brain cells until they die.
Andy B per 4 wrote @
1.)Tay-sachs disease is an inherited disease that is from the central nervous system. This disease effects babies. It occurs mostly in central and eastern european jews.
2.) Babies who have this disease appear healthy at birth and for the first months of their lives they develop normally. Symptoms appear at around 6 months. The baby stops smiling, crawling, turning over, and reaching out. The baby continues to lose skills and becomes blind and later paralyzed and unaware of the surroundings. Babies with this disease die around the age of five. There is currently no prognosis to stop the disease from affecting its victims.
3.)Babies that have Tay-sachs disease or Sandhoff disease lack an enzyme called hexosaminidase. There are two types of this disease, hex a and hex b. Babies with Tay-sachs do not make hex a and babies with sandhoff do not make hex a or hex b. Hexosaminidase is necessary for breaking down certain fatty substances in cells of the brain. Without this enzyme, the fatty substances build up and gradually destroy brain cells until the entire central nervous system stops working.
Jeff D period 3 wrote @
1. Tay- Sachs disease is an inherited disease that affects babies central nervous system that is fatal. Although not always, Tay- Sachs usually affects descendants of Central and Eastern European Jews babies. This occurs in a rate of about 1 in 600 for the Jews and 1 in 1000 for other ethnic groups.
2. The symptoms of Tay- Sachs disease for babies are as followed: when an supposably healthy baby slowly begins to stop smiling, crawling, turning over and reaching out. The prognosis for these babies with Tay- Sachs is that they will probably only live for 5 years.
3. Enzymes play a huge role in the origin of this disease. Babies with Tay- Sachs cannot make the enzyme hexosaminidase or hex A which is needed to break down fatty substances in the cells of the brain. This results in a buildup of these fatty substances which grows and destroys brain cells. This results in the destruction of the central nervous system and the death of the carrier.
Michelle R. wrote @
1. Tay-Sachs Disease is an inherited disease of the central nervous system. It occurs when harmful amounts of a fatty acid derivative called a gangioside (a lipid) collects in the nerve cells of the brain and a vital enzyme that breaks down lipids (Hexasaminidase A) has insufficient activity. This disease affects a child who has two parents that carries this genetic disorder.
2. The symptoms of Infantile Tay-Sachs Disease do not show until the child is about six months of age. The baby’s mental and physical abilities deteriorate relentlessly. They become blind, deaf, and unable to swallow. Their muscles begin to atrophy and paralysis sets in. Children with Infantile Tay-Sachs Disease die by the age of five. There are also two other forms of the disorder that are extremely rare; Juvenile T.S.D. and Adult/Late Onset T.S.D.
3. Tay-Sachs Disease is caused by the insufficient activity of the enzyme Hexosaminidase A (a vital hydrolytic enzyme found in the lysosomes that breaks down lipids) that catalyzes the biodegradation of fatty acid derivatives. When the enzyme is no longer functioning properly, the lipids accumulate in the brain and cause problems.
Chena Period 6 wrote @
1. Tay-Sachs disease is a genetic disease targeting infants and children whose parents are both carriers of the Tay-Sachs gene. Statistics show that Ashkenazi Jews who are from eastern Europe descent are at the highest risk for Tay-Sachs disease. Never the less, people throughout the world are diagnosed with this disease. A baby born with Tay-Sachs disease is born without on of the main enzymes, Hex A which is used to develop hearing, vision, movement, speech and other vital functions.
2. A baby who is born with Tay-Sachs appears to develop normally for the first sixth months of their life. From then on, they gradually begin to stop smiling, and crawling, then finally is paralyzed and blinded. Doctors predict that babies diagnosed with Tay-Sachs have until about the age of five to live.
3. Enzymes play a major role in this genetic disease, because the lack of the enzyme Hex A is the cause. The enzyme Hex A is used throughout the body to develop innate skills such as seeing, hearing, and smiling. The enzyme Hex A clears out unwanted material that can interfere with proper growth of these skills. Without this enzyme there is interference causing major problems.
Ellie – period 3 wrote @
1.Tay-Sachs disease is an inherited (genetic), incurable disease of the central nervous system. There is no cure for the disease and death usually occurs before the age of five years. Normally, what happens is that a baby about 6 months old begins to stop smiling and crawling, and loses other mental abilities. Not soon after these symptoms appear, the baby will begin to lose eye sight and hearing. Then the young child will become paralyzed. The reason the child becomes diagnosed from this terminal illness, is because the parents were carriers. So at birth, the child is lacking the correct genetic information to tell his/her body to produce an enzyme called Hexosaminidase A , which is used to break down certain substances in the brain and nerve cells. In most forms, it highly affects young children and babies; almost always at risk of death.
2.Tay-Sachs disease normally shows up at about the age of six months. Prior to that time, the baby acts normally. The main symptoms include loss of hearing, eyesight, and interactions with other people. The child may begin to stare and lose mental skills. Also, later into the stages of this terminal illness, the child will become paralyzed. Also one of the most disturbing and frightening symptoms is that the child lacks the ability to be aware of his/ her surroundings. The prognosis explains how this disease varies from infantile and juvenile forms that exhibit paralysis, dementia, blindness and early death. As you can see, over the course of this tragic disease, the symptoms become more and more severe.
3.Tay-Sachs disease is produced in the body due to the lack of an enzyme called Hexosaminidase A. This enzyme is important for breaking down certain fatty substances in cells of the brain. Without this enzyme, fatty substances build up and gradually destroy brain cells, until the entire central nervous system is destroyed. Basically, every enzyme in the body is important for different aspects. Although, all the enzymes revolve around the functions of other enzymes. With Tay-Sachs disease, the body is lacking Hexosaminidase A. This affects genetic disorder because lacking that enzyme causes malfunctions of information towards the brain and spinal cord. As a result, Tay-Sachs disease is severe and due to the lack of a specific enzyme, the body cannot continue working properly and therefore leads to death.
Ben wrote @
1. Tay- Sachs disease is inherited and affects the Central Nervous System. This disease is found in babies and is proven to be fatal. The “on-set” disease can develop between 2-10 years old, and prove fatal at age 15. Adult forms of the disease do exist in adolescence and sometimes in the 30’s. 1 out of every 30 American Jews carries a mutation in the gene that codes for Hex A, this does not mean that they will automatically receive the mutation. This mutation also occurs more in French- Canadian and Cajun peoples more often. People in other ethnic groups in this country have about a 1 in 300 chance of carrying a mutation in this gene.
2. Symptoms of Tay- Sachs disease usually begin 6 months after a baby is born. The baby will gradually stop smiling, rolling over, and reaching out. The baby will become blind and usually die around 5 years old. Adults with the disease may lose vision or hearing, have slurred speech, muscle weakness, muscle cramps, tremors, unsteady gait and sometimes mental illness. Life expectancy is around 4 to 5 years.
3. The enzyme hexosaminidase, breaks down the fatty substances that build up and gradually destroy brain cells. If Hex A or Hex B is not produced the substances spread until the entire central nervous system stops working. When the chromosomes 5 and/ or 15 are mutated so that Hex A or Hex B is not created, the child or person will be diagnosed with Tay- Sachs disease. The hexosaminidase speeds up the reaction of breaking down fatty substances, this way there is no excess build up in the brain.
Chelsea- period 4 wrote @
1. Tay-Sachs disease is an inherited disease throught the central nervous system. It is caused through genetic mutations in different genes. Tay-Sachs is a fatal disease that affects babies. These babies are born healthy and then begin to develop the symptoms at about 6 months of age.
2. Generally, after about the first few months of life, the symptoms of Tay-Sachs disease begin to appear in a baby. When a perfectly healthy baby stops smiling, crawling, turning over, and reaching out, a doctor starts to cosider that a baby might have Tay-Sachs disease. The symptoms continue to worsen as the baby becomes blind, paralyzed, and unaware of its surroundings. The prognosis for a baby with Tay-Sachs is it will most likely die by the age of 5.
3. Enzymes play a role in the genetic disorder because babies with Tay-Sachs disease lack an enzyme called hexosaminidase. Babies with this genetic disorder are unable to make one of the two versions of this enzyme (hex A and hex B), hex A. Some, but very few, babies with Tay-Sachs produce both of the versions of the enzyme, hexosaminidase, but fail to make a protein that is required for these enzymes to function properly. The enzyme, hexosaminidase is crucial because it breaks down certain fatty substances in the cells of a brain. If this enzyme is not present, then these fatty substances eventually build up, destroying brain cells until one’s entire central nervous system stops working.
Brooke r. wrote @
1. Tay-Sachs disease is an inherited disease of the central nervous system. it affects babies and it is fatal.
2. Babies with Tay-Sachs disease seem to be healthy when they are born and to be growing normally for the first few months of its life. Symptons from Tay-Sachs disease start to show at about the age of 6 months. A baby with Tay-Sachs disease starts to loose skills, soon becomes blind, paralyzed and unaware of their surroundings. They usually die by the age of 5 years old.
3. Babies with Tay-Sachs disease lack an enzyme called hexosaminidase. Hex A and hex B are two versions of the enzyme, and the babies often don’t make hex A. Some babies with Tay-Sachs have both enzymes, but lack another protein that is needed in order for the enzyne to work right.
Alex P. Period 6 wrote @
1. Tay-Sachs disease is a fatal disease which is inherited by the central nervous system. It is caused by mutations in different genes. Although this disease is generally found with babies, there is a form of Tay-Sachs disease called Juvenile, which affects people other than babies. Sadly, their is currently no treatment which will prevent Tay-Sachs disease from doing it’s job.
2.This disease has many different symptons. These different symptons generally occur about 6 months after the baby has been born
These symptoms include the following…
-baby stops smiling
-baby stops crawling
-baby stops turning over
-baby stops reaching out
-the baby will continue to lose skills gradually
In the long run a baby with this disease will eventually become paralyzed, go blind, and loose all awareness of its surroundings.
3.Hexosaminidase is one example of an enzyme. This enzyme is responsible for breaking down certain fatty substances called GM2 gangliosides- these are found in the cells of the brain. If this enzyme isn’t present then GM2 gangliosides build up and eventually destroy brain cells- leading to a collapse of the entire central nervous system.
Colby H. wrote @
1.Tay-Sachs disease is a disease that affects babies. It causes them to gradually lose skills until they are blind deaf and paralized.
2.Tay-Sachs disease causes babies to gradually stops smiling, crawling, turning over and reaching out. The babies continues to lose skills gradually and eventually becomes blind, paralyzed and unaware of their surroundings. The babies eventually die at age 5 or under.
3. Babies don’t have an enzyme called hexosaminidase which break down GM2 gangliosides these, if not broken down build up and kill brain cells.
Leah p4 wrote @
1. Tay-Sachs disease is a disease that is inherited and affects mainly infants. It is a disease in which an infant is missing an enzyme (hexosaminidase). This enzyme is the enzyme that breaks down fats in the brain that could potentially destroy brain cells. And with out it babies that have inherited it usually die around the age of 5, if not earlier.
2. For the classic Tay- Sachs disease (affecting infants) they are born and appear to be healthy. Around 6 months symptoms start to arise. The child loses skills like smiling, crawling, reaching up, turning over and things like that. The brain slowly shuts down and the baby loses its ability to do everything and can become paralyzed and blind. Children born with this disease usually die around the age of 5.
3. Enzymes are proteins that act as biological catalysts. They speed up chemical reactions throughout the body. The enzyme hexosaminidase is the enzyme in the brain that breaks down fats that can cause the brain damage until it can no longer function. Hexosaminidase is the enzyme missing in the disease Tay-Sachs. This causes the fat to remain in the brain because there is nothing to break it down and it slowly destroys the brains of infants/toddlers.
Stephen C. period 4 wrote @
1. Tay-Sachs diseases are a disease that destroys nerve cells in the brain and spinal cord. This is a very dangerous disease because it kills many infants at a young age. They usually die at the age of 5 if diagnosed with Tay-Sachs.
2. The symptoms of Tay-Sach disease are that many brain cells don’t work anymore. Eventually, the entire central nervous system stops working, and the child dies. It is a very dangerous disease, but very few of the population have it.
3. This disease works because of the lack of enzymes called hexosaminidase. A and B are two versions of this enzyme. Many babies don’t have hex A. Hexosaminidase is used for breaking down fatty substances, which are called GM2 gangliosides. Due to the lack of this enzyme, these fatty substances builds up in a child’s brain and destroys brain cells. The lack of brain cells leads to a complete fail in the entire central nervous system.
Brielle wrote @
1 it is a disease found in babies of eastern european decent. the disease effects the nervous system and it is the absence of an enzyme.
2 if your baby stops smiling crawling or turning over those are symptoms of it. if your baby is found to have this disease they have pretty much a guarenteed death by age 5
3 the absent enzyme is nessesary to brak down fatty substances and the fatty substances will build up in the brain and destroy brain cells and eventually the whole nervous system.
Stephanie M Period 6 wrote @
1. Tay-Sachs disease is an inherited disease of the central nervous system. It is caused by the changes in different genes. Tay-Sachs affects babies and it is fatal.
2. Infants at birth will seem healthy at first and for the first few months of living. At about 6 months of age a baby will stop smiling, crawling, and preforming other physical activity. The baby continues to lose skills gradually and becomes blind, paralyzed, and unaware of surroundings. They usually die at the age of five.
3. Babies with Tay-Sachs disease lack an enzyme called hexosaminidase. Hexosaminidase has two types, Hex A and Hex B. Hex A is not produced by babies with Tay-Sachs. Hexosaminidase is necessary for breaking down certain fatty substances in cells of the brain. Without it, fatty acids build up and destroy brain cell. Eventually the whole central nervous system stops working.
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1. Tay-Sachs disease is a inherited disease that affects the central nervous system. It affects babies and can be fatal.
2. Symptoms of Tay-Sachs can be seen 6 months after the baby is born. The baby will stop doing things it normally does and will continue to lose skills such as crawling and smiling. Eventually the baby will go blind, become paralyzed, and will be unaware of its surrounding until they die at around age 5.
3. The babies with Tay-Sachs disease lack the enzyme hexosaminidase. The body makes hex A and B. Tay-Sachs stops hex A from being made. Without hex A the body cannot break down fatty acids in the brain. These will build up until it destroys the brain cells and eventually destroy the entire central nervous system.