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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]
Gaucher disease is suggested based on the overall clinical picture. Initial laboratory testing may include enzyme testing. As a result, lower than 15% of mean normal activity is considered to be diagnostic. [23] Decreased enzyme levels will often be confirmed by genetic testing.
The specific activity of highly purified human enzyme is 890,000 units/mg, [5] meanwhile the enzyme activity produced by recombinant DNA technology is approximately 40 units/mg. [6] A typical dose is 2.5U/kg every two weeks, up to a maximum of 60 U/kg once every two weeks, and safety has been established from ages 2 and up. [7]
Alglucerase was a biopharmaceutical drug for the treatment of Gaucher's disease.It was a modified form of human β-glucocerebrosidase enzyme, where the non-reducing ends of the oligosaccharide chains have been terminated with mannose residues.
Enzyme activity can also be given as that of certain standardized substrates, such as gelatin, then measured in gelatin digesting units (GDU), or milk proteins, then measured in milk clotting units (MCU). The units GDU and MCU are based on how fast one gram of the enzyme will digest gelatin or milk proteins, respectively. 1 GDU approximately ...
A Georgia deputy fatally shot a Black man at point-blank range during a traffic stop after the man, who had been wrongfully imprisoned years ago, grabbed the officer by the neck and was forcing ...
As with many lipids, the chemical formula and molecular weight varies depending on the fatty-acid present. As one example, the chemical formula of Lactosylceramide (d18:1/12:0) is C 42 H 79 NO 13, which has 806.088 g/mol of molar mass, and the IUPAC name of this species is N-(dodecanoyl)-1-beta-lactosyl-sphing-4-enine.
The optimal pH for enzyme activity is 8.6. However, the activity is stable for a wide variety of conditions and reagents. PNGase F maintains 60% activity from pH 6.0 to pH 9.5. It is able to deglycosylate in the absence of denaturants, but needs extensive incubation and larger amounts of the enzyme to cleave native proteins. [1] [4] [5]