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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.
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
Trump “has made it clear that he expects these nations in the region to step up and to accept Palestinian refugees who are temporarily relocated for the rebuilding of Gaza,” White House press ...
President Donald Trump made a rare appearance with Elon Musk, his most powerful adviser, in the Oval Office on Tuesday before signing an executive order to continue downsizing the federal workforce.
The Minsk II agreement was negotiated in the Belarusian capital in 2015 in a bid to end a bloody 10-month conflict in eastern Ukraine. The rare diplomatic effort brought together leaders from ...
Taliglucerase alfa, sold under the brand name Elelyso among others, is a biopharmaceutical medication developed by Protalix and Pfizer. [3] [4] [full citation needed] The drug, a recombinant glucocerebrosidase used to treat Gaucher's disease, is the first plant-made pharmaceutical to win approval by the U.S. Food and Drug Administration (FDA).