Hexosaminidase (EC 3.2.1.52, β-acetylaminodeoxyhexosidase, N-acetyl-β-D-hexosaminidase, N-acetyl-β-hexosaminidase, N-acetyl hexosaminidase, β-hexosaminidase...
16 KB (1,464 words) - 17:02, 27 September 2023
gene on chromosome 15, which codes a subunit of the hexosaminidase enzyme known as hexosaminidase A. It is inherited in an autosomal recessive manner...
58 KB (6,401 words) - 02:47, 22 September 2024
Sandhoff disease (redirect from Total hexosaminidase deficiency)
disorder caused by the inherited deficiency to create functional beta-hexosaminidases A and B. These catabolic enzymes are needed to degrade the neuronal...
24 KB (3,024 words) - 14:53, 23 October 2024
Hexosaminidase A (alpha polypeptide), also known as HEXA, is an enzyme that in humans is encoded by the HEXA gene, located on the 15th chromosome. Hexosaminidase...
16 KB (2,014 words) - 09:28, 21 January 2024
Beta-hexosaminidase subunit beta is an enzyme that in humans is encoded by the HEXB gene. Hexosaminidase B is the beta subunit of the lysosomal enzyme...
13 KB (1,561 words) - 11:29, 18 August 2023
genetic disorders that result from a deficiency of the enzyme beta-hexosaminidase. This enzyme catalyzes the biodegradation of fatty acid derivatives...
12 KB (1,214 words) - 14:23, 12 October 2024
Infantile Juvenile Adult onset Tay–Sachs Juvenile hexosaminidase A deficiency Chronic hexosaminidase A deficiency Glucocerebroside Gaucher disease Type...
18 KB (1,831 words) - 14:49, 30 October 2024
Clostridium perfringens (section Hexosaminidase)
C. perfringens encodes for eight hexosaminidases that break down hexosamines in the mucus. These hexosaminidases belong to four glycoside hydrolase...
61 KB (6,856 words) - 23:30, 9 November 2024
In 2011, researchers discovered that pyrimethamine can increase β-hexosaminidase activity, thus potentially slowing down the progression of late-onset...
39 KB (3,121 words) - 17:53, 7 September 2024
substrate 4-nitrophenyl N-acetyl-β-D-glucosamine. Dispersin B is a β-hexosaminidase that specifically hydrolyzes β-1,6-glycosidic linkages of acetylglucosamine...
14 KB (1,639 words) - 21:02, 15 March 2024
same enzyme, beta-hexosaminidase. AB variant is caused by a failure in the gene that makes an enzyme cofactor for beta-hexosaminidase, called the GM2 activator...
8 KB (914 words) - 14:25, 12 October 2024
on chromosome 15. The HEXA gene makes part of an enzyme called beta-hexosaminidase A, which plays a critical role in the nervous system. This enzyme helps...
35 KB (4,371 words) - 03:38, 14 August 2024
are 3 forms of beta-hexosaminidase: hexosaminidase A is a trimer, with one alpha, one beta-A and one beta-B chain; hexosaminidase B is a tetramer of two...
5 KB (523 words) - 03:24, 9 February 2024
insufficiency is Tay–Sachs disease, in which patients lack the enzyme hexosaminidase. One example of enzyme deficiency is the most common type of phenylketonuria...
96 KB (9,821 words) - 16:54, 27 October 2024
Tay–Sachs disease arises as a genetic defect which leads to no functional hexosaminidase A produced, causing GM2 to accumulate in lysosomes. Ultimately the ganglion...
11 KB (1,307 words) - 21:11, 31 October 2024
sulfate proteoglycans adenosine triphosphate (ATP) lysosomal enzymes β-hexosaminidase β-glucuronidase arylsulfatases newly formed lipid mediators (eicosanoids):...
49 KB (5,615 words) - 06:31, 19 September 2024
enzymes are elevated, including tartrate-resistant acid phosphatase, hexosaminidase, and a human chitinase, chitotriosidase. This latter enzyme has proved...
30 KB (3,308 words) - 02:11, 3 November 2024
trait has been suggested. In 2008, researchers in Texas identified the hexosaminidase A deficiency known in humans as Tay–Sachs disease in four Jacob lambs...
31 KB (3,342 words) - 12:58, 24 September 2024
months of age. There is no cure for the disease. Mutations in the β-hexosaminidase A (Hex A) gene are known to affect the onset of Tay-Sachs, with 78 mutations...
36 KB (4,728 words) - 21:46, 4 October 2024
Ohno, Kousaku & Suzuki, Kunihiko (1988-12-05). "Multiple Abnormal beta-Hexosaminidase alpha-Chain mRNAs in a Compound-Heterozygous Ashkenazi Jewish Patient...
10 KB (1,252 words) - 19:17, 28 October 2024
evaluated by estimation of released lysosomal enzymes such as beta hexosaminidase. An electron-dense granule defined by the presence of Toll-like receptor...
14 KB (1,737 words) - 17:44, 17 September 2024
a substrate specific co-factor for the lysosomal enzyme β-hexosaminidase A. β-hexosaminidase A, together with GM2 ganglioside activator, catalyzes the...
10 KB (1,315 words) - 08:31, 18 August 2023
granzymes, lysosomal hydrolases (for example cathepsins B and D, β-hexosaminidase) and other cytolytic effector proteins. Once these proteins are delivered...
15 KB (1,808 words) - 03:31, 10 October 2024
to identify and purify a specific enzyme that cleaved hexosamine (a hexosaminidase) from a soil organism. Treating the polysaccharide with this enzyme...
13 KB (1,483 words) - 16:36, 3 December 2023
N-terminal domain represents the N-terminal domain in chitobiases and beta-hexosaminidases. Chitobiases degrade chitin, which forms the exoskeleton in insects...
2 KB (219 words) - 02:32, 23 December 2020
Actinobacillus actinomycetemcomitans biofilm cells by an endogenous beta-hexosaminidase activity". Journal of Bacteriology. 185 (16): 4693–8. doi:10.1128/JB...
121 KB (14,128 words) - 16:42, 9 October 2024
and BA3. Additionally, K252b dose-dependently inhibits Ag induced β-hexosaminidase release. K252b also inhibits IgE receptor-mediated degranulation of...
13 KB (1,334 words) - 10:47, 20 October 2024
Heterotaxy with polysplenia or asplenia Heterotaxy, visceral, X-linked Hexosaminidases A and B deficiency HHH syndrome Hibernian fever, familial Hiccups Hidradenitis...
21 KB (1,717 words) - 04:04, 12 March 2024
risks. Enzyme assay techniques detect individuals with lower levels of hexosaminidase A. Development of a serum enzyme assay test made it feasible to conduct...
19 KB (2,546 words) - 19:31, 22 June 2024
5-hydroxytryptophol, ethyl sulfate, phosphatidyl ethanol, sialic acid level, β-hexosaminidase, mitochondrial AST levels, and alcohol metabolites such as erythrocyte...
9 KB (929 words) - 13:26, 17 March 2024