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Galactosylceramidase (or galactocerebrosidase), EC 3.2.1.46, is an enzyme that removes galactose from ceramide derivatives (galactosylceramides) by catalysing the hydrolysis of galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. [1] It is a lysosomal protein, encoded in humans by ...
Glycoside hydrolase family 59 CAZY GH_59 comprises enzymes with only one known activity; galactocerebrosidase (EC 3.2.1.46). Globoid cell leukodystrophy (Krabbe disease) is a severe, autosomal recessive disorder that results from deficiency of galactocerebrosidase (GALC) activity.
β-Glucocerebrosidase (also called acid β-glucosidase, D-glucosyl-N-acylsphingosine glucohydrolase, or GCase) is an enzyme with glucosylceramidase activity (EC 3.2.1.45) that cleaves by hydrolysis the β-glycosidic linkage of the chemical glucocerebroside, an intermediate in glycolipid metabolism that is abundant in cell membranes (particularly skin cells). [5]
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These hydrogen bonds within the cerebrosides result in the molecules having a high transition temperature and compact alignment. Monoglycosylceramides in conjunction with cholesterol are prevalent in the lipid-raft micro domain, which are important sites in the binding of proteins, and enzyme-receptor interactions.
Krabbe disease (KD) (also known as globoid cell leukodystrophy [3] or galactosylceramide lipidosis) is a rare and often fatal lysosomal storage disease that results in progressive damage to the nervous system.
[24] [25] and, as a result, the mutant enzyme is able to replace the lacZ β-galactosidase. [26] EbgA and LacZ are 50% identical on the DNA level and 33% identical on the amino acid level. [27] The active ebg enzyme is an aggregate of ebgA -gene and ebgC-gene products in a 1:1 ratio with the active form of ebg enzymes being an α4 β4 hetero ...
Enzyme replacement therapy is available to treat mainly Fabry disease and Gaucher disease, and people with these types of sphingolipidoses may live well into adulthood. The other types are generally fatal by age 1 to 5 years for infantile forms, but progression may be mild for juvenile- or adult-onset forms.