Steroidogenic Acute Regulatory Protein (STAR)

STARD1; START domain-containing protein 1

SPN	Q291
HGNC	11359
MGI	102760
PubMed	17433772
RGD	3770
UniProt	P49675
Wiki	STAR

The steroidogenic acute regulatory protein is a transport protein that regulates cholesterol transfer within the mitochondria, which is the rate-limiting step in the production of steroid hormones. It is primarily present in steroid-producing cells, including theca cells and luteal cells in the ovary, Leydig cells in the testis and cell types in the adrenal cortex. The domain of StAR important for promoting cholesterol transfer is the StAR-related transfer domain (START domain). StAR is the prototypic member of the START domain family of proteins and is thus also known as STARD1 for "START domain-containing protein 1". It is hypothesized that the START domain forms a pocket in StAR that binds single cholesterol molecules for delivery to P450scc.

Reference

"Human steroidogenic acute regulatory protein: functional activity in COS-1 cells, tissue-specific expression, and mapping of the structural gene to 8p11.2 and a pseudogene to chromosome 13."
Proc. Natl. Acad. Sci. U.S.A. 92:4778-4782(1995) [PubMed] [Europe PMC] [Abstract]

"Structure of the human steroidogenic acute regulatory protein (StAR) gene: StAR stimulates mitochondrial cholesterol 27-hydroxylase activity."
Biochemistry 34:12506-12512(1995) [PubMed] [Europe PMC] [Abstract]

"The human steroidogenic acute regulatory (StAR) gene is expressed in the urogenital system and encodes a mitochondrial polypeptide."
Biochim. Biophys. Acta 1258:228-233(1995) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"The steroidogenic acute regulatory protein (StAR): a window into the complexities of intracellular cholesterol trafficking."
Recent Prog. Horm. Res. 54:369-394(1999) [PubMed] [Europe PMC] [Abstract]

"Comparative structural analysis of lipid binding START domains."
PLoS ONE 6:E19521-E19521(2011) [PubMed] [Europe PMC] [Abstract]

"The pathophysiology and genetics of congenital lipoid adrenal hyperplasia."
N. Engl. J. Med. 335:1870-1878(1996) [PubMed] [Europe PMC] [Abstract]

"Analysis of the steroidogenic acute regulatory protein (StAR) gene in Japanese patients with congenital lipoid adrenal hyperplasia."
Hum. Mol. Genet. 6:571-576(1997) [PubMed] [Europe PMC] [Abstract]

"A novel frameshift mutation 840delA and a novel polymorphism D203A in the steroidogenic acute regulatory protein gene in a Japanese patient with congenital lipoid adrenal hyperplasia."
Hum. Mutat. Suppl. 1:S304-S307(1998) [PubMed] [Europe PMC] [Abstract]

"A novel compound heterozygous mutation in the steroidogenic acute regulatory protein gene in a patient with congenital lipoid adrenal hyperplasia."
J. Clin. Endocrinol. Metab. 84:3983-3987(1999) [PubMed] [Europe PMC] [Abstract]