Target type: molecularfunction
Catalysis of the reaction: (24R,25R)-3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA = (24E)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA + H2O. [EC:4.2.1.107]
3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA hydratase activity catalyzes the hydration of the double bond in the 24-position of 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA, a key intermediate in the biosynthesis of bile acids. This enzyme is involved in the conversion of cholesterol to bile acids, a crucial process for lipid digestion and absorption. The reaction results in the formation of 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-oyl-CoA, which subsequently undergoes further enzymatic modifications to produce primary bile acids. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA hydratase activity is essential for maintaining cholesterol homeostasis and preventing its accumulation in the body. Deficiency in this activity can lead to cholestasis and other liver diseases.'
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| Protein | Definition | Taxonomy |
|---|---|---|
| Peroxisomal multifunctional enzyme type 2 | A peroxisomal multifunctional enzyme type 2 that is encoded in the genome of human. [PRO:DNx, UniProtKB:P51659] | Homo sapiens (human) |
| Compound | Definition | Classes | Roles |
|---|---|---|---|
| N-(4,6-dimethyl-2-pyridinyl)-4-[5-(trifluoromethyl)-2-pyridinyl]-1-piperazinecarbothioamide | piperazines; pyridines |