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Domain III (residues 76–381 and 416–430) is homologous to a TIM barrel and is a highly conserved domain among glycoside hydrolases. [8] Domain III harbors the active site, which binds the substrate glucocerebroside in close proximity to the catalytic residues E340 and E235. Domains I and III are tightly associated, while domains II and III ...
In Gaucher's disease, the enzyme glucocerebrosidase is nonfunctional and cannot break down glucocerebroside into glucose and ceramide in the lysosome. [1] Affected macrophages, called Gaucher cells, have a distinct appearance similar to "wrinkled tissue paper" under light microscopy, because the substrates build-up within the lysosome.
GD type II (acute infantile neuropathic) typically begins within six months of birth and has an incidence rate of around 1 in 100,000 live births. Symptoms include an enlarged liver and spleen, extensive and progressive brain damage, eye movement disorders, spasticity , seizures , limb rigidity, and a poor ability to suck and swallow.
DAVOS, SWITZERLAND — JPMorgan Chase (), like the rest of the world, is scrambling to figure out what the start of Trump 2.0 means.The largest US bank set up a "war room" to comb through all of ...
In enzymology, a glucosylceramidase (EC 3.2.1.45) is an enzyme that catalyzes the chemical reaction. D-glucosyl-N-acylsphingosine + H 2 O D-glucose + N-acylsphingosine. Thus, the two substrates of this enzyme are D-glucosyl-N-acylsphingosine and H 2 O, whereas its two products are D-glucose and N-acylsphingosine.
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John Daly is on the mend after receiving emergency hand surgery. On Tuesday, Jan. 14, the golfer, 58, shared a photo of himself on his Instagram Stories lying in a hospital bed, giving a thumbs-up ...
Ceramide glucosyltransferase (or glucosylceramide synthase) is a glucosyltransferase enzyme involved in the production of glucocerebrosides (also called glucosylceramides). ). It is responsible for the first step in synthesis of all glycosphingolipids—critical components of cell membranes—from sphingolipids, attaching a glucose molecule (glycosylation) to produce a glucocerebroside product