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.
Tay-Sachs disease is an autosomal recessive genetic disorder, meaning that when both parents are carriers, there is a 25% risk of giving birth to an affected child. As with all genetic disease, Tay-Sachs disease may arise in any generation from a novel mutation, although such mutations are rare.ay-Sachs disease is a rare, inherited disorder. It causes too much of a fatty substance to build up in tissues and nerve cells of the brain. This buildup destroys the nerve cells, causing mental and physical problems.