Incorrect folding of steroidogenic acute regulatory protein (StAR) in congenital lipoid adrenal hyperplasia

Biochemistry
H S BoseW L Miller

Abstract

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 StAR and four amino acid replacement/deletion mutants that cause lipoid CAH were purified to apparent homogeneity. Sedimentation equilibrium ultracentrifugation showed that all five proteins were monomeric and fit a globular protein model of the correct molecular mass. Circular dichroism (CD) spectra of both the wild-type and mutants showed minima near 208 and 222 nm, confirming the presence of substantial alpha-helical structure. However, subtle differences in the CD signals of the wild-type and mutants in the far-UV and stronger differences in near-UV indicated differences in protein folding. The amide I and II bands in the 1400-1700 cm-1 region of Fourier transform infrared spectra showed that the proteins fell into two groups. The wild-type and a partially active conservative mutant were predominantly alpha-helical with some intramolecular beta-sheet. By contrast, three mutants that lost charged residues retained much of their al...Continue Reading

Citations

Aug 26, 1999·BioEssays : News and Reviews in Molecular, Cellular and Developmental Biology·D M Stocco
Jul 27, 1999·The Journal of Steroid Biochemistry and Molecular Biology·W L Miller, J F Strauss
May 14, 1999·Trends in Biochemical Sciences·C P Ponting, L Aravind
Jun 17, 2000·Biochimica Et Biophysica Acta·D M Stocco
Dec 9, 2000·Biochimica Et Biophysica Acta·L K Christenson, J F Strauss
Feb 8, 2006·Canadian Journal of Microbiology·A J De LuccaD E Wedge
Jun 23, 1999·Proceedings of the National Academy of Sciences of the United States of America·H S BoseW L Miller
Dec 20, 2008·The Journal of Steroid Biochemistry and Molecular Biology·Elie BarbarJean-Guy Lehoux
Feb 26, 2008·Toxicology and Applied Pharmacology·Mahuya BoseHimangshu S Bose
Apr 17, 2004·BJU International·M NicolinoM David
May 28, 2003·Microscopy Research and Technique·Jean-Guy LehouxAlain Fleury
Dec 3, 2014·The Journal of Steroid Biochemistry and Molecular Biology·James L Thomas, Himangshu S Bose
Sep 16, 1999·Critical Reviews in Clinical Laboratory Sciences·C A Stratakis, O M Rennert
May 18, 2005·Journal of Lipid Research·Daniel Rodriguez-AgudoWilliam M Pandak
May 3, 2002·Nature·Himangshu S BoseWalter L Miller
Mar 30, 2001·The Journal of Biological Chemistry·K ChristensenJ D Bell
Oct 10, 2002·The Journal of Biological Chemistry·Robert C TuckeyWalter L Miller
Jan 18, 2003·Endocrine Research·Himangshu S BoseWalter L Miller
Oct 19, 2004·The Journal of Biological Chemistry·Dustin C YaworskyWalter L Miller

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