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steroidogenic acute regulatory protein, mitochondrial; StAR; StARD1 (STAR)
Function:
- role in movement of cholesterol across the outer mitochondrial membrane in steroidogenic cells for delivery to cyt P450scc (cholesterol 20,22 desmolase) within the inner mitochondrial membrane
- role in steroid hormone synthesis by enhancing metabolism of cholesterol into pregnenolone
- mediates the transfer of cholesterol from the outer mitochondrial membrane to the inner mitochondrial membrane where it is cleaved to pregnenolone
- steroid metabolism; cholesterol metabolism
- may interact with TSPO (putative)
Structure: contains 1 START domain
Compartment: mitochondria
Expression:
- expressed in gonads, adrenal cortex & kidney
Pathology:
- defects in STAR are a cause of adrenal hyperplasia 1
General
mitochondrial membrane protein
phosphoprotein
Properties
SIZE: entity length = 285 aa
MW = 32 kD
COMPARTMENT: outer mitochondrial membrane
MOTIF: Ser phosphorylation site {S57}
START {67-280}
MOTIF: Ser phosphorylation site {S195}
Database Correlations
OMIM correlations
MORBIDMAP 600617
UniProt P49675
Pfam PF01852
Entrez Gene 6770
Kegg hsa:6770
References
- Waterman MR.
A rising StAR: an essential role in cholesterol transport.
Science. 1995 Mar 24;267(5205):1780-1.
PMID: 7892600
- Lin D, Sugawara T, Strauss JF 3rd, Clark BJ, Stocco DM,
Saenger P, Rogol A, Miller WL.
Role of steroidogenic acute regulatory protein in adrenal and
gonadal steroidogenesis.
Science. 1995 Mar 24;267(5205):1828-31.
PMID: 7892608
- UniProt :accession P49675
- Wikipedia; steroidogenic acute regulatory protein entry
http://en.wikipedia.org/wiki/steroidogenic_acute_regulatory_protein
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=STAR