A0A0S2Z3R3 · A0A0S2Z3R3_HUMAN

  • Protein
    Cholesterol side-chain cleavage enzyme, mitochondrial
  • Gene
    CYP11A1
  • Status
    UniProtKB unreviewed (TrEMBL)
  • Amino acids
  • Protein existence
    Evidence at transcript level
  • Annotation score
    4/5

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.

Features

Showing features for binding site.

TypeIDPosition(s)Description
Binding site462Fe (UniProtKB | ChEBI) of heme (UniProtKB | ChEBI); axial binding residue

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 biosynthetic process
Biological Processcellular response to peptide hormone stimulus
Biological Processcholesterol metabolic process
Biological Processcortisol metabolic process
Biological Processglucocorticoid 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
  • Taxonomic lineage
    Eukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Mammalia > Eutheria > Euarchontoglires > Primates > Haplorrhini > Catarrhini > Hominidae > Homo

Accessions

  • Primary accession
    A0A0S2Z3R3

Organism-specific databases

Subcellular Location

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

Keywords

Disease & Variants

Organism-specific 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
    521
  • Mass (Da)
    60,102
  • Last updated
    2016-02-17 v1
  • Checksum
    AB0501E7A5665D8B
MLAKGLPPRSVLVKGCQTFLSAPREGLGRLRVPTGEGAGISTRSPRPFNEIPSPGDNGWLNLYHFWRETGTHKVHLHHVQNFQKYGPIYREKLGNVESVYVIDPEDVALLFKSEGPNPERFLIPPWVAYHQYYQRPIGVLLKKSAAWKKDRVALNQEVMAPEATKNFLPLLDAVSRDFVSVLHRRIKKAGSGNYSGDISDDLFRFAFESITNVIFGERQGMLEEVVNPEAQRFIDAIYQMFHTSVPMLNLPPDLFRLFRTKTWKDHVAAWDVIFSKADIYTQNFYWELRQKGSVHHDYRGILYRLLGDSKMSFEDIKANVTEMLAGGVDTTSMTLQWHLYEMARNLKVQDMLRAEVLAARHQAQGDMATMLQLVPLLKASIKETLRLHPISVTLQRYLVNDLVLRDYMIPAKTLVQVAIYALGREPTFFFDPENFDPTRWLSKDKNITYFRNLGFGWGVRQCLGRRIAELEMTIFLINMLENFRVEIQHLSDVGTTFNLILMPEKPISFTFWPFNQEATQQ

Features

Showing features for non-terminal residue.

TypeIDPosition(s)Description
Non-terminal residue521

Sequence databases

Nucleotide SequenceProtein SequenceMolecule TypeStatus
KU178011
EMBL· GenBank· DDBJ
ALQ33469.1
EMBL· GenBank· DDBJ
mRNA

Genome annotation databases

Similar Proteins

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