A0A087WRU6 · A0A087WRU6_MOUSE

  • Protein
    Cholesterol side-chain cleavage enzyme, mitochondrial
  • Gene
    Cyp11a1
  • Status
    UniProtKB unreviewed (TrEMBL)
  • Amino acids
  • Protein existence
    Evidence at protein level
  • Annotation score
    3/5

Function

function

A cytochrome P450 monooxygenase that catalyzes the side-chain hydroxylation and cleavage of cholesterol to pregnenolone, the precursor of most steroid hormones. Catalyzes three sequential oxidation reactions of cholesterol, namely the hydroxylation at C22 followed with the hydroxylation at C20 to yield 20R,22R-hydroxycholesterol that is further cleaved between C20 and C22 to yield the C21-steroid pregnenolone and 4-methylpentanal. Mechanistically, uses molecular oxygen inserting one oxygen atom into a substrate and reducing the second into a water molecule. Two electrons are provided by NADPH via a two-protein mitochondrial transfer system comprising flavoprotein FDXR (adrenodoxin/ferredoxin reductase) and nonheme iron-sulfur protein FDX1 or FDX2 (adrenodoxin/ferredoxin).

Catalytic activity

Cofactor

heme (UniProtKB | Rhea| CHEBI:30413 )

Pathway

Lipid metabolism; C21-steroid hormone metabolism.
Steroid metabolism; cholesterol metabolism.

GO annotations

AspectTerm
Cellular Componentmitochondrial inner membrane
Molecular Functioncholesterol monooxygenase (side-chain-cleaving) activity
Molecular Functionheme binding
Molecular Functioniron ion binding
Biological ProcessC21-steroid hormone metabolic process
Biological Processcholesterol metabolic process
Biological Processhormone biosynthetic process
Biological Processsteroid biosynthetic process

Keywords

Enzyme and pathway databases

Names & Taxonomy

Protein names

  • Recommended name
    Cholesterol side-chain cleavage enzyme, mitochondrial
  • EC number
  • Alternative names
    • Cholesterol desmolase

Gene names

    • Name
      Cyp11a1

Organism names

  • Taxonomic identifier
  • Organism
  • Strain
    • C57BL/6J
  • Taxonomic lineage
    Eukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Mammalia > Eutheria > Euarchontoglires > Glires > Rodentia > Myomorpha > Muroidea > Muridae > Murinae > Mus > Mus

Accessions

  • Primary accession
    A0A087WRU6

Proteomes

Organism-specific databases

Subcellular Location

Mitochondrion inner membrane
; Peripheral membrane protein
Note: Localizes to the matrix side of the mitochondrion inner membrane.

Keywords

PTM/Processing

Proteomic databases

Expression

Gene expression databases

Interaction

Subunit

Interacts with FDX1/adrenodoxin.

Family & Domains

Sequence similarities

Belongs to the cytochrome P450 family.

Keywords

Phylogenomic databases

Family and domain databases

Sequence

  • Sequence status
    Fragment
  • Length
    279
  • Mass (Da)
    32,127
  • Last updated
    2014-10-29 v1
  • Checksum
    0B683F40D9C3C2E6
MKEDGGREKVAYTCHNVQVQTQVHDTAVLKCKTEWRRSSDAWKKDRIVLNQEVMAPGAIKNFVPLLEGVAQDFIKVLHRRIKQQNSGNFSGVISDDLFRFSFESISSVIFGERMGMLEEIVDPEAQRFINAVYQMFHTSVPMLNLPPDFFRLLRTKTWKDHAAAWDVIFNKADEYTQNFYWDLRQKRDFSQYPGVLYSLLGGNKLPFKNIQANITEMLAGGVDTTSMTLQWNLYEMAHNLKVQEMLRAEVLAARRQAQGDMAKMVQLVPLLKASIKETL

Computationally mapped potential isoform sequences

There are 2 potential isoforms mapped to this entry

View all
EntryEntry nameGene nameLength
Q9QZ82CP11A_MOUSECyp11a1526
A0A087WRA8A0A087WRA8_MOUSECyp11a1115

Features

Showing features for non-terminal residue.

TypeIDPosition(s)Description
Non-terminal residue279

Keywords

Genome annotation databases

Similar Proteins

Disclaimer

Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Our staff consists of biologists and biochemists that are not trained to give medical advice.
We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.
FeedbackHelp