Author/Authors :
Lane K. Christenson، نويسنده , , Jerome F. StraussIII، نويسنده ,
DocumentNumber :
1601703
Title Of Article :
Steroidogenic acute regulatory protein (StAR) and the intramitochondrial translocation of cholesterol
شماره ركورد :
12055
Latin Abstract :
The steroidogenic acute regulatory (StAR) protein regulates the rate limiting step in steroidogenesis, the transport of cholesterol from the outer to the inner mitochondrial membrane. Insight into the structure and function of StAR was attained through molecular genetic studies of congenital lipoid adrenal hyperplasia, a rare disease caused by mutations in the StAR gene. Subsequent functional analysis defined two major domains within the StAR protein, the N-terminal mitochondrial targeting sequence and the C-terminus, which promotes the translocation of cholesterol between the two mitochondrial membranes. Two models of StAR’s mechanism of action, (1) stimulation of cholesterol desorption from the outer mitochondrial membrane and (2) an intermembrane shuttle hypothesis, are discussed with respect to the known biochemical and biophysical events associated with the process of steroidogenesis and the structure of StAR. StAR gene expression is regulated primarily at the transcriptional level, and the roles of transcription factors that govern basal and cAMP-dependent StAR expression including SF-1, C/EBP β, Sp1 and GATA-4 are reviewed.
From Page :
175
NaturalLanguageKeyword :
transcription , Cholesterol , Steroidogenic acute regulatory protein , mitochondria , steroidogenesis
JournalTitle :
Studia Iranica
To Page :
187
To Page :
187
Link To Document :
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