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Chem."},{"key":"10.1194\/jlr.R016675_bib81","doi-asserted-by":"crossref","first-page":"33357","DOI":"10.1074\/jbc.M802686200","article-title":"Identification of neutral cholesterol ester hydrolase, a key enzyme removing cholesterol from macrophages","volume":"283","author":"Okazaki","year":"2008","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib82","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1016\/j.bbrc.2010.11.103","article-title":"Abrogation of neutral cholesterol ester hydrolytic activity causes adrenal enlargement","volume":"404","author":"Ohta","year":"2011","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1194\/jlr.R016675_bib83","doi-asserted-by":"crossref","first-page":"2713","DOI":"10.1172\/JCI116888","article-title":"A splice junction mutation causes deletion of a 72-base exon from the mRNA for lysosomal acid lipase in a patient with cholesteryl ester storage disease","volume":"92","author":"Klima","year":"1993","journal-title":"J. Clin. Invest."},{"key":"10.1194\/jlr.R016675_bib84","doi-asserted-by":"crossref","first-page":"2718","DOI":"10.1073\/pnas.91.7.2718","article-title":"Mutations at the lysosomal acid cholesteryl ester hydrolase gene locus in Wolman disease","volume":"91","author":"Anderson","year":"1994","journal-title":"Proc. Natl. Acad. Sci. USA."},{"key":"10.1194\/jlr.R016675_bib85","doi-asserted-by":"crossref","first-page":"1382","DOI":"10.1016\/S0022-2275(20)32518-9","article-title":"New lysosomal acid lipase gene mutants explain the phenotype of Wolman disease and cholesteryl ester storage disease","volume":"39","author":"Pagani","year":"1998","journal-title":"J. Lipid Res."},{"key":"10.1194\/jlr.R016675_bib86","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1016\/S0022-2275(20)33360-5","article-title":"Molecular defects underlying Wolman disease appear to be more heterogeneous than those resulting in cholesteryl ester storage disease","volume":"40","author":"Lohse","year":"1999","journal-title":"J. Lipid Res."},{"key":"10.1194\/jlr.R016675_bib87","doi-asserted-by":"crossref","first-page":"1033","DOI":"10.1016\/S0022-2275(20)31591-1","article-title":"Characterization of lysosomal acid lipase mutations in the signal peptide and mature polypeptide region causing Wolman disease","volume":"42","author":"Zschenker","year":"2001","journal-title":"J. Lipid Res."},{"key":"10.1194\/jlr.R016675_bib88","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/j.ymgme.2009.02.007","article-title":"Cholesteryl ester storage disease (CESD) due to novel mutations in the LIPA gene","volume":"97","author":"Pisciotta","year":"2009","journal-title":"Mol. Genet. Metab."},{"key":"10.1194\/jlr.R016675_bib89","doi-asserted-by":"crossref","first-page":"3243","DOI":"10.1210\/jc.2004-0016","article-title":"Primary adrenal insufficiency in children: twenty years experience at the Sainte-Justine Hospital, Montreal","volume":"90","author":"Perry","year":"2005","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"10.1194\/jlr.R016675_bib90","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1007\/s00431-006-0298-6","article-title":"Successful treatment of Wolman disease by unrelated umbilical cord blood transplantation","volume":"166","author":"Stein","year":"2007","journal-title":"Eur. J. 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Natl. Acad. Sci. USA."},{"key":"10.1194\/jlr.R016675_bib94","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1210\/me.2003-0179","article-title":"Hormone-sensitive lipase is required for HDL cholestyryl ester-supported adrenal steroidogenesis","volume":"18","author":"Kraemer","year":"2004","journal-title":"Mol. Endocrinol."},{"key":"10.1194\/jlr.R016675_bib95","doi-asserted-by":"crossref","first-page":"1010","DOI":"10.1194\/jlr.M300009-JLR200","article-title":"Trafficking defects in endogenously synthesized cholesterol in fibroblasts, macrophages, hepatocytes and glial cells from Niemann-Pick type C1 mice","volume":"44","author":"Reid","year":"2003","journal-title":"J. Lipid Res."},{"key":"10.1194\/jlr.R016675_bib96","doi-asserted-by":"crossref","first-page":"20917","DOI":"10.1074\/jbc.R400040200","article-title":"Niemann-Pick type C disease and intracellular cholesterol trafficking","volume":"280","author":"Chang","year":"2005","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib97","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1034\/j.1399-0004.2003.00147.x","article-title":"Niemann-Pick disease type C","volume":"64","author":"Vanier","year":"2003","journal-title":"Clin. Genet."},{"key":"10.1194\/jlr.R016675_bib98","doi-asserted-by":"crossref","first-page":"1739","DOI":"10.1212\/WNL.45.9.1739","article-title":"Psychosis as the initial manifestation of adult-onset Niemann-Pick disease type C","volume":"45","author":"Shulman","year":"1995","journal-title":"Neurology."},{"key":"10.1194\/jlr.R016675_bib99","doi-asserted-by":"crossref","first-page":"56ra81","DOI":"10.1126\/scitranslmed.3001417","article-title":"Cholesterol oxidation products are sensitive and specific blood-based biomarkers for Niemann-Pick C1 disease","volume":"2","author":"Porter","year":"2010","journal-title":"Sci. Transl. 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Chem."},{"key":"10.1194\/jlr.R016675_bib102","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1007\/s004390050370","article-title":"A C57BL\/KsJ mouse model of Niemann-Pick disease (spm) belongs to the same complementation group as the major childhood type of Niemann-Pick disease type C","volume":"99","author":"Akaboshi","year":"1997","journal-title":"Hum. Genet."},{"key":"10.1194\/jlr.R016675_bib103","doi-asserted-by":"crossref","first-page":"6245","DOI":"10.1074\/jbc.272.10.6245","article-title":"Peroxisomal impairment in Niemann-Pick type C disease","volume":"272","author":"Schedin","year":"1997","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib104","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1007\/BF00578243","article-title":"A molecular genetic linkage map of mouse chromosome 18, including spm, Grl-1, Fim-2\/c-fms, and Mbp","volume":"29","author":"Sakai","year":"1991","journal-title":"Biochem. 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Chem."},{"key":"10.1194\/jlr.R016675_bib120","doi-asserted-by":"crossref","first-page":"765","DOI":"10.1016\/S1474-4422(07)70194-1","article-title":"Miglustat for treatment of Niemann-Pick C disease: a randomised controlled study","volume":"6","author":"Patterson","year":"2007","journal-title":"Lancet Neurol."},{"key":"10.1194\/jlr.R016675_bib121","doi-asserted-by":"crossref","first-page":"704","DOI":"10.1038\/nm1073","article-title":"Niemann-Pick type C disease involves disrupted neurosteroidogenesis and responds to allopregnanolone","volume":"10","author":"Griffin","year":"2004","journal-title":"Nat. Med."},{"key":"10.1194\/jlr.R016675_bib122","doi-asserted-by":"crossref","first-page":"811","DOI":"10.1002\/jnr.20685","article-title":"Allopregnanolone treatment, both as a single injection or repetitively, delays demyelination and enhances survival of Niemann-Pick C mice","volume":"82","author":"Ahmad","year":"2005","journal-title":"J. Neurosci. 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Endocrinol."},{"key":"10.1194\/jlr.R016675_bib180","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1016\/j.bbalip.2011.06.028","article-title":"Subcellular localization and regulation of StarD4 protein in macrophages and fibroblasts","volume":"1811","author":"Rodriguez-Agudo","year":"2011","journal-title":"Biochim. Biophys. Acta."},{"key":"10.1194\/jlr.R016675_bib181","doi-asserted-by":"crossref","first-page":"13731","DOI":"10.1073\/pnas.93.24.13731","article-title":"Steroidogenic acute regulatory protein (StAR) retains activity in the absence of its mitochondrial targeting sequence: Implications for the mechanism of StAR action","volume":"93","author":"Arakane","year":"1996","journal-title":"Proc. Natl. Acad. Sci. 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USA."},{"key":"10.1194\/jlr.R016675_bib185","doi-asserted-by":"crossref","first-page":"5153","DOI":"10.1016\/S0021-9258(18)92004-1","article-title":"On the mechanism of action of adrenocorticotropic hormone. The inhibitory site of cycloheximide in the pathway of steroid biosynthesis","volume":"243","author":"Davis","year":"1968","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib186","doi-asserted-by":"crossref","first-page":"1590","DOI":"10.1073\/pnas.84.6.1590","article-title":"Coordinate tropic hormone regulation of mRNAs for insulin-like growth factor II and the cholesterol side-chain cleavage enzyme, P450scc, in human steroidogenic tissues","volume":"84","author":"Voutilainen","year":"1987","journal-title":"Proc. Natl. Acad. Sci. 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Chem."},{"key":"10.1194\/jlr.R016675_bib193","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1095\/biolreprod.104.037721","article-title":"Involvement of protein kinase C and cyclic adenosine 3\u2032,5\u2032-monophosphate-dependent kinase in steroidogenic acute regulatory protein expression and steroid biosynthesis in Leydig cells","volume":"73","author":"Jo","year":"2005","journal-title":"Biol. Reprod."},{"key":"10.1194\/jlr.R016675_bib194","doi-asserted-by":"crossref","first-page":"2647","DOI":"10.1210\/me.2004-0532","article-title":"Multiple signaling pathways regulating steroidogenesis and steroidogenic acute regulatory protein expression: more complicated than we thought","volume":"19","author":"Stocco","year":"2005","journal-title":"Mol. Endocrinol."},{"key":"10.1194\/jlr.R016675_bib195","doi-asserted-by":"crossref","first-page":"6594","DOI":"10.1016\/S0021-9258(19)84604-5","article-title":"Acute stimulation of steroidogenesis in corpus luteum and adrenal cortex by peptide hormones","volume":"261","author":"Pon","year":"1986","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib196","doi-asserted-by":"crossref","first-page":"13309","DOI":"10.1016\/S0021-9258(18)69305-6","article-title":"Acute ACTH regulation of adrenal corticosteroid biosynthesis: rapid accumulation of a phosphoprotein","volume":"261","author":"Pon","year":"1986","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib197","doi-asserted-by":"crossref","first-page":"19739","DOI":"10.1016\/S0021-9258(18)55054-7","article-title":"Regulation of steroid hormone biosynthesis: identification of precursors of a phosphoprotein targeted to the mitochondrion in stimulated rat adrenal cortex cells","volume":"266","author":"Epstein","year":"1991","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib198","doi-asserted-by":"crossref","first-page":"19731","DOI":"10.1016\/S0021-9258(18)55053-5","article-title":"The 30 kDa mitochondrial protein induced by hormone stimulation in MA-10 mouse Leydig tumor cells are processed from larger precursors","volume":"266","author":"Stocco","year":"1991","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib199","doi-asserted-by":"crossref","first-page":"28314","DOI":"10.1016\/S0021-9258(18)46930-X","article-title":"The purification, cloning and expression of a novel luteinizing hormone-induced mitochondrial protein in MA-10 mouse Leydig tumor cells. Characterization of the steroidogenic acute regulatory protein (StAR)","volume":"269","author":"Clark","year":"1994","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib200","doi-asserted-by":"crossref","first-page":"1828","DOI":"10.1126\/science.7892608","article-title":"Role of steroidogenic acute regulatory protein in adrenal and gonadal steroidogenesis","volume":"267","author":"Lin","year":"1995","journal-title":"Science."},{"key":"10.1194\/jlr.R016675_bib201","doi-asserted-by":"crossref","first-page":"1870","DOI":"10.1056\/NEJM199612193352503","article-title":"The pathophysiology and genetics of congenital lipoid adrenal hyperplasia","volume":"335","author":"Bose","year":"1996","journal-title":"N. Engl. J. 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Chem."},{"key":"10.1194\/jlr.R016675_bib228","doi-asserted-by":"crossref","first-page":"1267","DOI":"10.1073\/pnas.98.3.1267","article-title":"Cholesterol binding at the cholesterol recognition\/interaction amino acid consensus (CRAC) of the peripheral-type benzodiazepine receptor and inhibition of steroidogenesis by an HIV TAT-CRAC peptide","volume":"98","author":"Li","year":"2001","journal-title":"Proc. Natl. Acad. Sci. USA."},{"key":"10.1194\/jlr.R016675_bib229","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1146\/annurev.pa.32.040192.001235","article-title":"Mitochondrial benzodiazapine receptors and the regulation of steroid biosynthesis","volume":"32","author":"Krueger","year":"1992","journal-title":"Annu. Rev. Pharmacol. 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Chem."},{"key":"10.1194\/jlr.R016675_bib243","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1136\/adc.30.154.538","article-title":"A form of lipoidosis of the adrenal cortex in an infant","volume":"30","author":"Sandison","year":"1955","journal-title":"Arch. Dis. Child."},{"key":"10.1194\/jlr.R016675_bib244","first-page":"569","article-title":"Adrenal insufficiency in congenital lipoid hyperplasia of the adrenals","volume":"12","author":"Prader","year":"1957","journal-title":"Helv. Paediatr. Acta."},{"key":"10.1194\/jlr.R016675_bib245","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1210\/jcem-36-3-488","article-title":"Congenital lipoid adrenal hyperplasia in an eight-year-old phenotypic female","volume":"36","author":"Kirkland","year":"1973","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"10.1194\/jlr.R016675_bib246","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1111\/j.1365-2265.1985.tb01107.x","article-title":"Congenital adrenal hyperplasia due to deficient cholesterol side-chain cleavage activity (20,22 desmolase) in a patient treated for 18 years","volume":"23","author":"Hauffa","year":"1985","journal-title":"Clin. Endocrinol. (Oxf.)."},{"key":"10.1194\/jlr.R016675_bib247","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1210\/jcem-28-2-153","article-title":"Congenital adrenal hyperplasia due to a deficiency of one of the enzymes involved in the biosynthesis of pregnenolone","volume":"28","author":"Camacho","year":"1968","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"10.1194\/jlr.R016675_bib248","doi-asserted-by":"crossref","first-page":"512","DOI":"10.1530\/acta.0.0710512","article-title":"Evidence for deficiency of 20\u03b1 cholesterol hydroxylase activity in adrenal tissue of a patient with lipoid adrenal hyperplasia","volume":"71","author":"Degenhart","year":"1972","journal-title":"Acta Endocrinol. (Copenh.)."},{"key":"10.1194\/jlr.R016675_bib249","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1016\/0009-8981(77)90233-9","article-title":"Cholesterol side-chain cleavage enzyme activity and cytochrome P450 content in adrenal mitochondria of a patient with congenital lipoid adrenal hyperplasia (Prader disease)","volume":"77","author":"Koizumi","year":"1977","journal-title":"Clin. Chim. Acta."},{"key":"10.1194\/jlr.R016675_bib250","doi-asserted-by":"crossref","first-page":"1296","DOI":"10.1210\/endo-118-4-1296","article-title":"Study of cholesterol side chain cleavage (20,22 desmolase) deficiency causing congenital lipoid adrenal hyperplasia using bovine-sequence P450scc oligodeoxyribonucleotide probes","volume":"118","author":"Matteson","year":"1986","journal-title":"Endocrinology."},{"key":"10.1194\/jlr.R016675_bib251","doi-asserted-by":"crossref","first-page":"1955","DOI":"10.1172\/JCI115520","article-title":"Normal genes for the cholesterol side chain cleavage enzyme, P450scc, in congenital lipoid adrenal hyperplasia","volume":"88","author":"Lin","year":"1991","journal-title":"J. Clin. Invest."},{"key":"10.1194\/jlr.R016675_bib252","first-page":"200","article-title":"Prenatal diagnosis of congenital lipoid adrenal hyperplasia","volume":"80","author":"Saenger","year":"1995","journal-title":"J. Clin. Endocrinol. 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Endocrinol."},{"key":"10.1194\/jlr.R016675_bib255","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1016\/S0960-0760(98)00153-8","article-title":"Molecular pathology and mechanism of action of the steroidogenic acute regulartory protein, StAR","volume":"69","author":"Miller","year":"1999","journal-title":"J. Steroid Biochem. Mol. Biol."},{"key":"10.1194\/jlr.R016675_bib256","doi-asserted-by":"crossref","first-page":"2299","DOI":"10.1093\/hmg\/4.12.2299","article-title":"T\u2192A transversion 11 bp from a splice acceptor site in the gene for steroidogenic acute regulatory protein causes congenital lipoid adrenal hyperplasia","volume":"4","author":"Tee","year":"1995","journal-title":"Hum. Mol. 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Endocrinol."},{"key":"10.1194\/jlr.R016675_bib261","doi-asserted-by":"crossref","first-page":"835","DOI":"10.1210\/jc.2004-1323","article-title":"A genetic isolate of congenital lipoid adrenal hyperplasia with atypical clinical findings","volume":"90","author":"Chen","year":"2005","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"10.1194\/jlr.R016675_bib262","doi-asserted-by":"crossref","first-page":"1145","DOI":"10.1210\/jcem-63-5-1145","article-title":"Developmental expression of genes for the steroidogenic enzymes P450scc (20,22 desmolase), P450c17 (17\u03b1-hydroxylase\/17,20 lyase) and P450c21 (21-hydroxylase) in the human fetus","volume":"63","author":"Voutilainen","year":"1986","journal-title":"J. Clin. Endocrinol. 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Metab."},{"key":"10.1194\/jlr.R016675_bib269","doi-asserted-by":"crossref","first-page":"4781","DOI":"10.1210\/jc.2006-1565","article-title":"Non-classic congenital lipoid adrenal hyperplasia: a new disorder of the steroidogenic acute regulatory protein with very late presentation and normal male genitalia","volume":"91","author":"Baker","year":"2006","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"10.1194\/jlr.R016675_bib270","doi-asserted-by":"crossref","first-page":"3865","DOI":"10.1210\/jc.2009-0467","article-title":"Nonclassic lipoid congenital adrenal hyperplasia masquerading as familial glucocorticoid deficiency","volume":"94","author":"Metherell","year":"2009","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"10.1194\/jlr.R016675_bib271","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1016\/S0031-6997(25)00021-3","article-title":"The molecular biology of cytochrome P450s","volume":"40","author":"Gonzalez","year":"1988","journal-title":"Pharmacol. Rev."},{"key":"10.1194\/jlr.R016675_bib272","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1016\/S0378-1119(00)00533-3","article-title":"The cytochrome P450 gene superfamily in Drosophila melanogaster: annotation, intron-exon organization and phylogeny","volume":"262","author":"Tijet","year":"2001","journal-title":"Gene."},{"key":"10.1194\/jlr.R016675_bib273","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1097\/00008571-200401000-00001","article-title":"Comparison of cytochrome P450 (CYP) genes from the mouse and human genomes, including nomenclature recommendations for genes, pseudogenes and alternative-splice variants","volume":"14","author":"Nelson","year":"2004","journal-title":"Pharmacogenetics."},{"key":"10.1194\/jlr.R016675_bib274","doi-asserted-by":"crossref","first-page":"7104","DOI":"10.1073\/pnas.85.19.7104","article-title":"Human adrenodoxin reductase: Two mRNAs encoded by a single gene of chromosome 17cen \u2192 q25 are expressed in steroidogenic tissues","volume":"85","author":"Solish","year":"1988","journal-title":"Proc. Natl. Acad. Sci. USA."},{"key":"10.1194\/jlr.R016675_bib275","doi-asserted-by":"crossref","first-page":"3240","DOI":"10.1016\/S0021-9258(18)69061-1","article-title":"Human adrenodoxin: cloning of three cDNAs and cycloheximide enhancement in JEG-3 cells","volume":"263","author":"Picado-Leonard","year":"1988","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib276","doi-asserted-by":"crossref","unstructured":"276., Pawlak, K. J., Prasad, M., Thomas, J. L., Whittal, R. M., Bose, H. S., . 2011. Inner mitochondrial translocase Tim50 interacts with 3beta-hydroxysteroid dehydrogenase type-2 to regulate adrenal and gonadal steroidogenesis. J. Biol. Chem. Epub ahead of print. September 19, 2011; doi:.","DOI":"10.1074\/jbc.M111.290031"},{"key":"10.1194\/jlr.R016675_bib277","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/0003-9861(55)90014-X","article-title":"Studies on ACTH action in perfused bovine adrenals: aspects of progesterone as an intermediary in cortico-steroidogenesis","volume":"54","author":"Stone","year":"1955","journal-title":"Arch. Biochem. Biophys."},{"key":"10.1194\/jlr.R016675_bib278","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1016\/S0021-9258(18)64370-4","article-title":"A requirement for reduced triphosphopyridine nucleotide for cholesterol side-chain cleavage by mitochondrial fractions of bovine adrenal cortex","volume":"236","author":"Halkerston","year":"1961","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib279","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1016\/S0021-9258(18)63433-7","article-title":"On the mechanism of action of adrenocorticotropic hormone - the stimulation of the activity of enzymes involved in pregnenolone synthesis","volume":"245","author":"Koritz","year":"1970","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib280","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1016\/0039-128X(86)90002-4","article-title":"Cytochromes P450 and the regulation of steroid syntheses","volume":"48","author":"Hall","year":"1986","journal-title":"Steroids."},{"key":"10.1194\/jlr.R016675_bib281","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1111\/j.1432-1033.1993.tb18235.x","article-title":"Side-chain specificities of human and bovine cytochromes P-450scc","volume":"217","author":"Tuckey","year":"1993","journal-title":"Eur. J. Biochem."},{"key":"10.1194\/jlr.R016675_bib282","doi-asserted-by":"crossref","first-page":"1785","DOI":"10.1210\/endo-111-6-1785","article-title":"Metabolism of 25-hydroxycholesterol by rat luteal mitochondria and dispersed cells","volume":"111","author":"Toaff","year":"1982","journal-title":"Endocrinology."},{"key":"10.1194\/jlr.R016675_bib283","doi-asserted-by":"crossref","first-page":"1501","DOI":"10.1016\/0006-291X(91)90457-I","article-title":"Purification and properties of cytochrome P-450(SCC) from pig testis mitochondria","volume":"176","author":"Kuwada","year":"1991","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1194\/jlr.R016675_bib284","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1210\/edrv-9-3-295","article-title":"Molecular biology of steroid hormone synthesis","volume":"9","author":"Miller","year":"1988","journal-title":"Endocr. 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Biochem."},{"key":"10.1194\/jlr.R016675_bib290","doi-asserted-by":"crossref","first-page":"1977","DOI":"10.1210\/endo.132.5.7682938","article-title":"Inherited congenital adrenal hyperplasia in the rabbit is caused by a deletion in the gene encoding cytochrome P450 cholesterol side-chain cleavage enzyme","volume":"132","author":"Yang","year":"1993","journal-title":"Endocrinology."},{"key":"10.1194\/jlr.R016675_bib291","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1016\/S0006-291X(02)02280-5","article-title":"Altered profile of gene expression in rat hearts induced by nicotine consumption","volume":"297","author":"Hu","year":"2002","journal-title":"Biochem. Biophys. Res. 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Chem."},{"key":"10.1194\/jlr.R016675_bib302","doi-asserted-by":"crossref","first-page":"39144","DOI":"10.1074\/jbc.M205786200","article-title":"The transcriptional regulating protein of 132 kDa (TReP-132) enhances P450scc gene transcription through interaction with steroidogenic factor-1 in human adrenal cells","volume":"277","author":"Gizard","year":"2002","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib303","doi-asserted-by":"crossref","first-page":"5704","DOI":"10.1016\/S0021-9258(18)83606-7","article-title":"Comparison of pregnenolone synthesis by cytochrome P-450scc in mitochondria from porcine corpora lutea and granulosa cells of follicles","volume":"264","author":"Tuckey","year":"1989","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib304","doi-asserted-by":"crossref","first-page":"4591","DOI":"10.1242\/dev.126.20.4591","article-title":"The dare gene: steroid hormone production, olfactory behaviour, and neural degradation in drosophila","volume":"126","author":"Freeman","year":"1999","journal-title":"Development."},{"key":"10.1194\/jlr.R016675_bib305","doi-asserted-by":"crossref","first-page":"8516","DOI":"10.1073\/pnas.87.21.8516","article-title":"Cloning and sequence of the human adrenodoxin reductase gene","volume":"87","author":"Lin","year":"1990","journal-title":"Proc. Natl. Acad. Sci. USA."},{"key":"10.1194\/jlr.R016675_bib306","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1089\/dna.1991.10.359","article-title":"Regional mapping of genes encoding human steroidogenic enzymes: P450scc to 15q23-q24, adrenodoxin to 11q22; adrenodoxin reductase to 17q24-q25; and P450c17 to 10q24-q25","volume":"10","author":"Sparkes","year":"1991","journal-title":"DNA Cell Biol."},{"key":"10.1194\/jlr.R016675_bib307","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1016\/0003-9861(92)90749-M","article-title":"Expression and characterization of human mitochondrial ferredoxin reductase in Escherichia coli","volume":"294","author":"Brandt","year":"1992","journal-title":"Arch. Biochem. Biophys."},{"key":"10.1194\/jlr.R016675_bib308","doi-asserted-by":"crossref","first-page":"351","DOI":"10.3892\/ol_00000062","article-title":"Influence of 5-fluorouracil on ferredoxin reductase mRNA splice variants in colorectal carcinomas","volume":"1","author":"Schneider","year":"2010","journal-title":"Oncology Lett."},{"key":"10.1194\/jlr.R016675_bib309","doi-asserted-by":"crossref","first-page":"4099","DOI":"10.1073\/pnas.89.9.4099","article-title":"cAMP post-transcriptionally diminishes the abundance of adrenodoxin reductase mRNA","volume":"89","author":"Brentano","year":"1992","journal-title":"Proc. Natl. Acad. Sci. USA."},{"key":"10.1194\/jlr.R016675_bib310","doi-asserted-by":"crossref","first-page":"981","DOI":"10.1006\/jmbi.1999.2807","article-title":"The structure of adrenodoxin reductase of mitochondrial P450 systems: electron transfer for steroid biosynthesis","volume":"289","author":"Ziegler","year":"1999","journal-title":"J. Mol. Biol."},{"key":"10.1194\/jlr.R016675_bib311","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1016\/S0039-128X(96)00170-5","article-title":"Molecular recognition and electron transfer in mitochondrial steroid hydroxylase systems","volume":"62","author":"Vickery","year":"1997","journal-title":"Steroids."},{"key":"10.1194\/jlr.R016675_bib312","doi-asserted-by":"crossref","first-page":"17126","DOI":"10.1016\/S0021-9258(19)85311-5","article-title":"Charge pair interactions stabilizing ferredoxin-ferredoxin reductase complexes: identification by complementary site-specific mutations","volume":"268","author":"Brandt","year":"1993","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib313","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1089\/dna.1988.7.609","article-title":"Cloning and structure of the human adrenodoxin gene","volume":"7","author":"Chang","year":"1988","journal-title":"DNA."},{"key":"10.1194\/jlr.R016675_bib314","doi-asserted-by":"crossref","first-page":"1373","DOI":"10.1083\/jcb.111.4.1373","article-title":"Induction and mitochondrial localization of cytochrome P450scc system enzymes in normal and transformed ovarian granulosa cells","volume":"111","author":"Hanukoglu","year":"1990","journal-title":"J. Cell Biol."},{"key":"10.1194\/jlr.R016675_bib315","doi-asserted-by":"crossref","first-page":"11775","DOI":"10.1073\/pnas.1004250107","article-title":"Humans possess two mitochondrial ferredoxins, Fdx1 and Fdx2, with distinct roles in steroidogenesis, heme, and Fe\/S cluster biosynthesis","volume":"107","author":"Sheftel","year":"2010","journal-title":"Proc. Natl. Acad. Sci. 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Chem."},{"key":"10.1194\/jlr.R016675_bib318","doi-asserted-by":"crossref","first-page":"22877","DOI":"10.1016\/S0021-9258(18)50028-4","article-title":"Identification by site-directed mutagenesis of two lysine residues in cholesterol side chain cleavage cytochrome P450 that are essential for adrenodoxin binding","volume":"267","author":"Wada","year":"1992","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.R016675_bib319","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1016\/S0167-4838(99)00243-5","article-title":"Construction and characterization of a catalytic fusion protein system: P-450(11beta)-adrenodoxin reductase-adrenodoxin","volume":"1476","author":"Cao","year":"2000","journal-title":"Biochim. Biophys. 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