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.
Metabolic refers to the body's chemical processes that produce protein and other substances, and break down nutrients to release energy. Tay-Sachs disease is a metabolic disorder because it is caused by the absence of the enzyme (a type of protein) hexosaminidase A (Hex-A). Hex-A is necessary for breaking down fatty substances called lipids. Without Hex-A, these lipids build up in, and eventually destroy, the nerve cells in the brain.