hexosaminidase造句

• Functional lysosomal ?-hexosaminidase enzymes are dimeric in structure.
• In particular, beta-hexosaminidase A breaks down a fatty compound called GM2 ganglioside.
• Tay & ndash; Sachs disease occurs when hexosaminidase A loses its ability to function.
• Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases.
• Beta-hexosaminidase is a vital hydrolytic enzyme, found in the lysosomes, that breaks down lipids.
• Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes.
• There are numerous mutations that lead to hexosaminidase A deficiency including gene deletions, nonsense mutations, and missense mutations.
• Some lysosomal enzymes are elevated, including tartrate-resistant acid phosphatase, hexosaminidase, and a human chitinase, chitotriosidase.
• Researchers have also tried directly instilling the deficient enzyme hexosaminidase A into the cerebrospinal fluid ( CSF ) which bathes the brain.
• This swelling is shown, for instance, in Tay-Sachs, a GM2 accumulation due to defective Beta-Hexosaminidase.
• A fourth mammalian hexosaminidase polypeptide which has been designated hexosaminidase D ( " HEXDC " ) has recently been identified.
• A fourth mammalian hexosaminidase polypeptide which has been designated hexosaminidase D ( " HEXDC " ) has recently been identified.
• When beta-hexosaminidase is no longer functioning properly, the lipids accumulate in the nervous tissue of the brain and cause problems.
• An example of a fatal genetic disease due to enzyme insufficiency is Tay-Sachs disease, in which patients lack the enzyme hexosaminidase.
• If you read the article below, you will find that the exact molecular role of beta-hexosaminidase A is not even known.
• If mutations in both alleles at this locus disrupt the activity of the GM2 activator, beta-hexosaminidase A cannot perform its normal function.
• This protein is a cofactor that is required for the normal function of beta-hexosaminidase A . The disease is usually fatal by early childhood.
• Beta-hexosaminidase and the cofactor G M2 activator protein catalyze the degradation of the G M2 gangliosides and other molecules containing terminal N-acetyl hexosamines.
• Even though the alpha and beta subunits of hexosaminidase A can both cleave GalNAc residues, only the alpha subunit is able to hydrolyze G M2 gangliosides.
• In order to prove this specificity, he first had to identify and purify a specific enzyme that cleaved hexosamine ( a hexosaminidase ) from a soil organism.