Whereas the content standards will provide the topic of the lesson and what exactly the students should be doing with that topic (e.g., solving problems, creating models, ranking ideas), the English language proficiency or development standards help to identify language skills and functions that students should be working on to achieve academic language fluency.
Tay-Sachs disease is a gangliosidosis characterised by: An exaggerated startle response. Delay in psychomotor development. Hypotonia (followed by spasticity) Visual loss. A macular cherry-red spot. Pathogenesis. Tay-Sachs disease (and its variants) are caused by absence or defects of the alpha subunit of hexosaminidase A.
But a baby with Tay-Sachs disease is born without one of those important enzymes, hexosaminidase A (HEXA). So, as those fatty proteins build up in the brain, they hurt the baby's sight, hearing, movement, and mental development. A child can only get Tay-Sachs by inheriting the gene for it from both parents. Tay-Sachs can be detected before birth, so couples who are thinking of having children may want to get a blood test to find out whether their child would be likely to have it. Who Is at Risk for Tay-Sachs? Each year, about 16 cases of Tay-Sachs are diagnosed in the United States.