EC - 3β-hydroxysteroid-4α-carboxylate 3-dehydrogenase (decarboxylating)

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IntEnz Enzyme Nomenclature


Accepted name:
3β-hydroxysteroid-4α-carboxylate 3-dehydrogenase (decarboxylating)
Other names:
3β-hydroxy-4β-methylcholestenecarboxylate 3-dehydrogenase (decarboxylating)
3β-hydroxy-4β-methylcholestenoate dehydrogenase
sterol 4α-carboxylic decarboxylase
3β-hydroxy-4α-methylcholestenecarboxylate 3-dehydrogenase (decarboxylating)
sterol-4α-carboxylate 3-dehydrogenase (decarboxylating) [ambiguous]
ERG26 (gene name)
NSDHL (gene name)
Systematic name:
3β-hydroxysteroid-4α-carboxylate:NAD(P)+ 3-oxidoreductase (decarboxylating)



The enzyme catalyses the decarboxylation of the C-4 carbon and the dehydrogenation of a 3β hydroxyl at the C-3 carbon of 3β-hydroxysteroid-4α-carboxylates. It is involved in zymosterol and cholesterol biosynthesis.

Links to other databases

Enzymes and pathways: NC-IUBMB , BRENDA , DIAGRAM , ExplorEnz , ENZYME@ExPASy , KEGG , MetaCyc , UniPathway
Structural data: CSA , EC2PDB
Gene Ontology: GO:0103066 , GO:0103067 , GO:0047012
CAS Registry Number: 71822-23-6
UniProtKB/Swiss-Prot: (10) [show] [UniProt]


  1. Brady, D.R., Crowder, R.D. and Hayes, W.J.
    Mixed function oxidases in sterol metabolism. Source of reducing equivalents.
    J. Biol. Chem. 255: 10624-10629 (1980). [PMID: 7430141]
  2. Rahimtula, A.D. and Gaylor, J.L.
    Partial purification of a microsomal sterol 4α-carboxylic acid decarboxylase.
    J. Biol. Chem. 247: 9-15 (1972). [PMID: 4401584]
  3. Gachotte, D., Barbuch, R., Gaylor, J., Nickel, E., Bard, M.
    Characterization of the Saccharomyces cerevisiae ERG26 gene encoding the C-3 sterol dehydrogenase (C-4 decarboxylase) involved in sterol biosynthesis.
    Proc. Natl. Acad. Sci. U.S.A. 95: 13794-13799 (1998). [PMID: 9811880]
  4. Caldas, H., Herman, G. E.
    NSDHL, an enzyme involved in cholesterol biosynthesis, traffics through the Golgi and accumulates on ER membranes and on the surface of lipid droplets.
    Hum. Mol. Genet. 12: 2981-2991 (2003). [PMID: 14506130]

[EC created 1978, modified 2002, modified 2012]