Incorrect Folding of Steroidogenic Acute Regulatory Protein (StAR) in Congenital Lipoid Adrenal Hyperplasia

• Published in: Biochemistry (Easton) Vol. 37; no. 27; pp. 9768 - 9775
• Main Authors: Bose, Himangshu S, Baldwin, Michael A, Miller, Walter L
• Format: Journal Article
• Language: English
• Published: United States American Chemical Society 07.07.1998
• Subjects: Adrenal Hyperplasia, Congenital - genetics; Adrenal Hyperplasia, Congenital - metabolism; Circular Dichroism; Escherichia coli - genetics; Guanidine - pharmacology; Hot Temperature; Humans; Mutagenesis, Site-Directed; Phosphoproteins - chemistry; Phosphoproteins - genetics; Phosphoproteins - isolation & purification; Protein Denaturation - drug effects; Protein Folding; Recombinant Proteins - biosynthesis; Recombinant Proteins - chemistry; Recombinant Proteins - isolation & purification; Spectroscopy, Fourier Transform Infrared; Urea - pharmacology

Steroidogenic acute regulatory protein (StAR) rapidly stimulates the movement of cholesterol into adrenal and gonadal mitochondria to mediate the acute steroidogenic response; StAR mutations cause potentially lethal congenital lipoid adrenal hyperplasia (lipoid CAH). Bacterially expressed wild-type...