(25R)-cholest-5-en-26-oic acid, 3,7-hydroxy

Uses

3β,7β-dihydroxy-5-cholestenoic acid may be used as an authentic diastereoisomer standard in mass spectrometry (MS) experiments.

General Description

3β,7β-dihydroxy-5-cholestenoic acid (3β,7β-diHCA) is associated with plasma and is a C27 acid. 3β,7β-diHCA is synthesized from 3β-hydroxy-7-oxocholest-5-en-(25R)26-oic acid (3βH,7O-CA) by the action of the enzyme hydroxysteroid 11-β dehydrogenase 1.

Biochem/physiol Actions

3β,7β-dihydroxy-5-cholestenoic acid (3β,7β-diHCA) levels are elevated in Niemann-Pick type C (NPC) disease and Niemann-Pick (NP) disease type B disorders. It elicits toxicity to oculomotor neurons.