- Display observed secondary structure variance per amino acid residue
Cystathionine beta-synthase
CBS Enzyme: EC 4.2.1.22 This enzyme has known catalytic activity involved in reaction: L-homocysteine + L-serine = L,L-cystathionine + H2O Disease Cystathionine beta-synthase deficiency: An enzymatic deficiency resulting in altered sulfur metabolism and homocystinuria. The clinical features of untreated homocystinuria due to CBS deficiency include myopia, ectopia lentis, intellectual disability, skeletal anomalies resembling Marfan syndrome, and thromboembolic events... [click for more]
Homo sapiens (Human)
P35520go to UniProt
Hydro-lyase catalyzing the first step of the transsulfuration pathway, where the hydroxyl group of L-serine is displaced by L-homocysteine in a beta-replacement reaction to form L-cystathionine, the precursor of L-cysteine. This catabolic route allows the elimination of L-methionine and the toxic metabolite L-homocysteine (PubMed:20506325, PubMed:23974653, PubMed:23981774). Also involved in the production ... [show more]go to UniProt
Representative structures for UniProt P35520
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PDB chain shown: 1jbq A go to PDBe
UniProt residues 1 - 413
Coverage: 63%