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Science 24 March 1995:
Vol. 267. no. 5205, pp. 1828 - 1831
DOI: 10.1126/science.7892608

Articles

Science, Vol 267, Issue 5205, 1828-1831
Copyright © 1995 by American Association for the Advancement of Science


articles

Role of steroidogenic acute regulatory protein in adrenal and gonadal steroidogenesis

D Lin, T Sugawara, JF Strauss 3rd, BJ Clark, DM Stocco, P Saenger, A Rogol, and WL Miller

Department of Pediatrics, University of California, San Francisco 94143.

Congenital lipoid adrenal hyperplasia is an autosomal recessive disorder that is characterized by impaired synthesis of all adrenal and gonadal steroid hormones. In three unrelated individuals with this disorder, steroidogenic acute regulatory protein, which enhances the mitochondrial conversion of cholesterol into pregnenolone, was mutated and nonfunctional, providing genetic evidence that this protein is indispensable normal adrenal and gonadal steroidogenesis.


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Mol. Endocrinol. 16, 2297-2309
   Abstract »    Full Text »    PDF »
Regulation of Steroidogenic Genes by Insulin-Like Growth Factor-1 and Follicle-Stimulating Hormone: Differential Responses of Cytochrome P450 Side-Chain Cleavage, Steroidogenic Acute Regulatory Protein, and 3{beta}-Hydroxysteroid Dehydrogenase/Isomerase in Rat Granulosa Cells.
S. Eimerl and J. Orly (2002)
Biol Reprod 67, 900-910
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Compound Heterozygous Mutations in the Cholesterol Side-Chain Cleavage Enzyme Gene (CYP11A) Cause Congenital Adrenal Insufficiency in Humans.
N. Katsumata, M. Ohtake, T. Hojo, E. Ogawa, T. Hara, N. Sato, and T. Tanaka (2002)
J. Clin. Endocrinol. Metab. 87, 3808-3813
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Monogenic Disorders of Puberty.
S. N. Kalantaridou and G. P. Chrousos (2002)
J. Clin. Endocrinol. Metab. 87, 2481-2494
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Crystal structure of the Mus musculus cholesterol-regulated START protein 4 (StarD4) containing a StAR-related lipid transfer domain.
M. J. Romanowski, R. E. Soccio, J. L. Breslow, and S. K. Burley (2002)
PNAS 99, 6949-6954
   Abstract »    Full Text »    PDF »
Oxytocin Receptor Regulation and Action in a Human Granulosa-Lutein Cell Line.
J. A. Copland, M. G. Zlatnik, K. L. Ives, and M. S. Soloff (2002)
Biol Reprod 66, 1230-1236
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Autoimmune Adrenocortical Failure in Norway Autoantibodies and Human Leukocyte Antigen Class II Associations Related to Clinical Features.
A. G. Myhre, D. E. Undlien, K. Lovas, S. Uhlving, B. G. Nedrebo, K. J. Fougner, T. Trovik, J. I. Sorheim, and E. S. Husebye (2002)
J. Clin. Endocrinol. Metab. 87, 618-623
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Interaction Between Dax-1 and Steroidogenic Factor-1 in Vivo: Increased Adrenal Responsiveness to ACTH in the Absence of Dax-1.
P. S. Babu, D. L. Bavers, F. Beuschlein, S. Shah, B. Jeffs, J. L. Jameson, and G. D. Hammer (2002)
Endocrinology 143, 665-673
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Regulation of Steroidogenesis and the Steroidogenic Acute Regulatory Protein by a Member of the cAMP Response-Element Binding Protein Family.
P. R. Manna, M. T. Dyson, D. W. Eubank, B. J. Clark, E. Lalli, P. Sassone-Corsi, A. J. Zeleznik, and D. M. Stocco (2002)
Mol. Endocrinol. 16, 184-199
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Mitochondrial Processing of Newly Synthesized Steroidogenic Acute Regulatory Protein (StAR), but Not Total StAR, Mediates Cholesterol Transfer to Cytochrome P450 Side Chain Cleavage Enzyme in Adrenal Cells.
I. P. Artemenko, D. Zhao, D. B. Hales, K. H. Hales, and C. R. Jefcoate (2001)
J. Biol. Chem. 276, 46583-46596
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Recent Advances in Membrane Microdomains: Rafts, Caveolae, and Intracellular Cholesterol Trafficking.
F. Schroeder, A. M. Gallegos, B. P. Atshaves, S. M. Storey, A. L. McIntosh, A. D. Petrescu, H. Huang, O. Starodub, H. Chao, H. Yang, et al. (2001)
Experimental Biology and Medicine 226, 873-890
   Abstract »    Full Text »    PDF »
The OSBP-related protein family in humans.
M. Lehto, S. Laitinen, G. Chinetti, M. Johansson, C. Ehnholm, B. Staels, E. Ikonen, and V. M. Olkkonen (2001)
J. Lipid Res. 42, 1203-1213
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Heterozygous Mutation in the Cholesterol Side Chain Cleavage Enzyme (P450scc) Gene in a Patient with 46,XY Sex Reversal and Adrenal Insufficiency.
T. Tajima, K. Fujieda, N. Kouda, J. Nakae, and W. L. Miller (2001)
J. Clin. Endocrinol. Metab. 86, 3820-3825
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Tracking the Role of a StAR in the Sky of the New Millennium.
D. M. Stocco (2001)
Mol. Endocrinol. 15, 1245-1254
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Salt-Inducible Kinase Is Involved in the ACTH/cAMP-Dependent Protein Kinase Signaling in Y1 Mouse Adrenocortical Tumor Cells.
X.-z. Lin, H. Takemori, Y. Katoh, J. Doi, N. Horike, A. Makino, Y. Nonaka, and M. Okamoto (2001)
Mol. Endocrinol. 15, 1264-1276
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Decreased Expression of Steroidogenic Acute Regulatory Protein: A Novel Mechanism Participating in the Leptin-Induced Inhibition of Glucocorticoid Biosynthesis.
N. Cherradi, A. M. Capponi, R. C. Gaillard, and F. P. Pralong (2001)
Endocrinology 142, 3302-3308
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RIP 140 Modulates Transcription of the Steroidogenic Acute Regulatory Protein Gene through Interactions with Both SF-1 and DAX-1.
T. Sugawara, S. Abe, N. Sakuragi, Y. Fujimoto, E. Nomura, K. Fujieda, M. Saito, and S. Fujimoto (2001)
Endocrinology 142, 3570-3577
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Lipoid Congenital Adrenal Hyperplasia (CAH): Patient Report and a Mini-Review.
E. J. Richmond, C. J. Flickinger, J. A. McDonald, M. A. Lovell, and A. D. Rogol (2001)
Clinical Pediatrics 40, 403-407
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Leukemia Inhibitory Factor Antagonizes Gonadotropin Induced-Testosterone Synthesis in Cultured Porcine Leydig Cells: Sites of Action.
C. Mauduit, I. Goddard, V. Besset, E. Tabone, C. Rey, F. Gasnier, F. Dacheux, and M. Benahmed (2001)
Endocrinology 142, 2509-2520
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Creation and Activity of COS-1 Cells Stably Expressing the F2 Fusion of the Human Cholesterol Side-Chain Cleavage Enzyme System.
M.-C. Huang and W. L. Miller (2001)
Endocrinology 142, 2569-2576
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Ectopic and Abnormal Hormone Receptors in Adrenal Cushing's Syndrome.
A. Lacroix, N. N'Diaye, J. Tremblay, and P. Hamet (2001)
Endocr. Rev. 22, 75-110
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