{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T08:51:02Z","timestamp":1792399862397,"version":"4.4.0"},"reference-count":23,"publisher":"American Association for the Advancement of Science (AAAS)","issue":"5205","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Science"],"published-print":{"date-parts":[[1995,3,24]]},"abstract":"<jats:p>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 for normal adrenal and gonadal steroidogenesis.<\/jats:p>","DOI":"10.1126\/science.7892608","type":"journal-article","created":{"date-parts":[[2006,10,27]],"date-time":"2006-10-27T14:19:42Z","timestamp":1161958782000},"page":"1828-1831","source":"Crossref","is-referenced-by-count":834,"title":["Role of Steroidogenic Acute Regulatory Protein in Adrenal and Gonadal Steroidogenesis"],"prefix":"10.1126","volume":"267","author":[{"given":"Dong","family":"Lin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teruo","family":"Sugawara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"III","given":"Jerome F.","family":"Strauss","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara J.","family":"Clark","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Douglas M.","family":"Stocco","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Saenger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan","family":"Rogol","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Walter L.","family":"Miller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"221","reference":[{"key":"e_1_2_1_1_1","doi-asserted-by":"crossref","first-page":"28314","DOI":"10.1016\/S0021-9258(18)46930-X","volume":"269","year":"1994","unstructured":"CLARK, B.J., 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), JOURNAL OF BIOLOGICAL CHEMISTRY 269: 28314 (1994).","journal-title":"JOURNAL OF BIOLOGICAL CHEMISTRY"},{"key":"e_1_2_1_2_1","doi-asserted-by":"crossref","first-page":"8144","DOI":"10.1016\/S0021-9258(19)70620-6","volume":"255","year":"1980","unstructured":"CRIVELLO, J.F., INTRACELLULAR MOVEMENT OF CHOLESTEROL IN RAT ADRENAL-CELLS - KINETICS AND EFFECTS OF INHIBITORS, JOURNAL OF BIOLOGICAL CHEMISTRY 255: 8144 (1980).","journal-title":"JOURNAL OF BIOLOGICAL CHEMISTRY"},{"key":"e_1_2_1_3_1","first-page":"512","volume":"71","year":"1972","unstructured":"Degenhart, H. J., Acta Endocrinologica 71: 512 (1972).","journal-title":"Acta Endocrinologica"},{"key":"e_1_2_1_4_1","doi-asserted-by":"crossref","first-page":"19739","DOI":"10.1016\/S0021-9258(18)55054-7","volume":"266","year":"1991","unstructured":"EPSTEIN, L.F., REGULATION OF STEROID-HORMONE BIOSYNTHESIS - IDENTIFICATION OF PRECURSORS OF A PHOSPHOPROTEIN TARGETED TO THE MITOCHONDRION IN STIMULATED RAT ADRENAL-CORTEX CELLS, JOURNAL OF BIOLOGICAL CHEMISTRY 266: 19739 (1991).","journal-title":"JOURNAL OF BIOLOGICAL CHEMISTRY"},{"key":"e_1_2_1_5_1","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1089\/dna.1993.12.371","volume":"12","year":"1993","unstructured":"HARIKRISHNA, J.A., CONSTRUCTION AND FUNCTION OF FUSION ENZYMES OF THE HUMAN CYTOCHROME-P450SCC SYSTEM, DNA AND CELL BIOLOGY 12: 371 (1993).","journal-title":"DNA AND CELL BIOLOGY"},{"key":"e_1_2_1_6_1","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1111\/j.1365-2265.1985.tb01107.x","volume":"23","year":"1985","unstructured":"HAUFFA, B.P., CONGENITAL ADRENAL-HYPERPLASIA DUE TO DEFICIENT CHOLESTEROL SIDE-CHAIN CLEAVAGE ACTIVITY (20, 22-DESMOLASE) IN A PATIENT TREATED FOR 18 YEARS, CLINICAL ENDOCRINOLOGY 23: 481 (1985).","journal-title":"CLINICAL ENDOCRINOLOGY"},{"key":"e_1_2_1_7_1","doi-asserted-by":"crossref","first-page":"721","DOI":"10.1016\/0022-4731(87)90142-7","volume":"27","year":"1987","unstructured":"JEFCOATE, C.R., ACTH REGULATION OF CHOLESTEROL MOVEMENT IN ISOLATED ADRENAL-CELLS, JOURNAL OF STEROID BIOCHEMISTRY AND MOLECULAR BIOLOGY 27: 721 (1987).","journal-title":"JOURNAL OF STEROID BIOCHEMISTRY AND MOLECULAR BIOLOGY"},{"key":"e_1_2_1_8_1","first-page":"4715","volume":"83","year":"1986","unstructured":"JOHN, M.E., TRANSCRIPTIONAL REGULATION OF STEROID HYDROXYLASE GENES BY CORTICOTROPIN, PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA 83: 4715 (1986).","journal-title":"PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA"},{"key":"e_1_2_1_9_1","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1016\/0009-8981(77)90233-9","volume":"77","year":"1977","unstructured":"KOIZUMI, S, CHOLESTEROL SIDE-CHAIN CLEAVAGE ENZYME-ACTIVITY AND CYTOCHROME-P-450 CONTENT IN ADRENAL MITOCHONDRIA OF A PATIENT WITH CONGENITAL LIPOID ADRENAL-HYPERPLASIA (PRADER DISEASE), CLINICA CHIMICA ACTA 77: 301 (1977).","journal-title":"CLINICA CHIMICA ACTA"},{"key":"e_1_2_1_10_1","first-page":"1249","volume":"6","year":"1992","unstructured":"LALA, D.S., STEROIDOGENIC FACTOR-I, A KEY REGULATOR OF STEROIDOGENIC ENZYME EXPRESSION, IS THE MOUSE HOMOLOG OF FUSHI-TARAZU-FACTOR-1, MOLECULAR ENDOCRINOLOGY 6: 1249 (1992).","journal-title":"MOLECULAR ENDOCRINOLOGY"},{"key":"e_1_2_1_11_1","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1016\/S0888-7543(05)80367-2","volume":"18","year":"1993","unstructured":"LIN, D, THE HUMAN PERIPHERAL BENZODIAZEPINE RECEPTOR GENE - CLONING AND CHARACTERIZATION OF ALTERNATIVE SPLICING IN NORMAL-TISSUES AND IN A PATIENT WITH CONGENITAL LIPOID ADRENAL-HYPERPLASIA, GENOMICS 18: 643 (1993).","journal-title":"GENOMICS"},{"key":"e_1_2_1_12_1","doi-asserted-by":"crossref","first-page":"1955","DOI":"10.1172\/JCI115520","volume":"88","year":"1991","unstructured":"LIN, D, NORMAL GENES FOR THE CHOLESTEROL SIDE-CHAIN CLEAVAGE ENZYME, P450SCC, IN CONGENITAL LIPOID ADRENAL-HYPERPLASIA, JOURNAL OF CLINICAL INVESTIGATION 88: 1955 (1991).","journal-title":"JOURNAL OF CLINICAL INVESTIGATION"},{"key":"e_1_2_1_13_1","unstructured":"LIN D unpublished data."},{"key":"e_1_2_1_14_1","first-page":"1003","volume":"78","year":"1994","unstructured":"MELLON, S.H., SPECIAL ARTICLE - NEUROSTEROIDS - BIOCHEMISTRY, MODES OF ACTION, AND CLINICAL RELEVANCE, JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM 78: 1003 (1994).","journal-title":"JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM"},{"key":"e_1_2_1_15_1","first-page":"6013","volume":"10","year":"1990","unstructured":"MOORE, CCD, HUMAN-P450SCC GENE-TRANSCRIPTION IS INDUCED BY CYCLIC-AMP AND REPRESSED BY 12-O-TETRADECANOYLPHORBOL-13-ACETATE AND A23187 THROUGH INDEPENDENT CIS ELEMENTS, MOLECULAR AND CELLULAR BIOLOGY 10: 6013 (1990).","journal-title":"MOLECULAR AND CELLULAR BIOLOGY"},{"key":"e_1_2_1_16_1","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/1043-2760(94)90114-7","volume":"5","year":"1994","unstructured":"ROBEL, P, TRENDS IN ENDOCRINOLOGY AND METABOLISM 5: 1 (1994).","journal-title":"TRENDS IN ENDOCRINOLOGY AND METABOLISM"},{"key":"e_1_2_1_17_1","first-page":"200","volume":"80","year":"1995","unstructured":"SAENGER, P, PRENATAL-DIAGNOSIS OF CONGENITAL LIPOID ADRENAL-HYPERPLASIA, JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM 80: 200 (1995).","journal-title":"JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM"},{"key":"e_1_2_1_18_1","first-page":"1198","volume":"79","year":"1994","unstructured":"SAKAI, Y, NO MUTATION IN CYTOCHROME-P450 SIDE-CHAIN CLEAVAGE IN A PATIENT WITH CONGENITAL LIPOID ADRENAL-HYPERPLASIA, JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM 79: 1198 (1994).","journal-title":"JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM"},{"key":"e_1_2_1_19_1","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1210\/endo.132.3.8382603","volume":"132","year":"1993","unstructured":"STOCCO, D.M., THE USE OF GENETIC MANIPULATION OF MA-10 LEYDIG TUMOR-CELLS TO DEMONSTRATE THE ROLE OF MITOCHONDRIAL PROTEINS IN THE ACUTE REGULATION OF STEROIDOGENESIS, ENDOCRINOLOGY 132: 959 (1993).","journal-title":"ENDOCRINOLOGY"},{"key":"e_1_2_1_20_1","doi-asserted-by":"crossref","first-page":"19731","DOI":"10.1016\/S0021-9258(18)55053-5","volume":"266","year":"1991","unstructured":"STOCCO, D.M., THE 30-KDA MITOCHONDRIAL PROTEINS INDUCED BY HORMONE STIMULATION IN MA-10 MOUSE LEYDIG TUMOR-CELLS ARE PROCESSED FROM LARGER PRECURSORS, JOURNAL OF BIOLOGICAL CHEMISTRY 266: 19731 (1991).","journal-title":"JOURNAL OF BIOLOGICAL CHEMISTRY"},{"key":"e_1_2_1_21_1","unstructured":"Strauss J. F. Endocrinology 3 3 : 2171 (1995)."},{"key":"e_1_2_1_22_1","first-page":"4778","volume":"92","year":"1995","unstructured":"Sugawara, T., Proceedings of the National Academy of Sciences of the United States of America 92: 4778 (1995).","journal-title":"Proceedings of the National Academy of Sciences of the United States of America"},{"key":"e_1_2_1_23_1","doi-asserted-by":"crossref","first-page":"1785","DOI":"10.1210\/endo-111-6-1785","volume":"111","year":"1982","unstructured":"TOAFF, M.E., METABOLISM OF 25-HYDROXYCHOLESTEROL BY RAT LUTEAL MITOCHONDRIA AND DISPERSED CELLS, ENDOCRINOLOGY 111: 1785 (1982).","journal-title":"ENDOCRINOLOGY"}],"container-title":["Science"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.science.org\/doi\/pdf\/10.1126\/science.7892608","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,14]],"date-time":"2024-01-14T23:25:05Z","timestamp":1705274705000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.science.org\/doi\/10.1126\/science.7892608"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1995,3,24]]},"references-count":23,"journal-issue":{"issue":"5205","published-print":{"date-parts":[[1995,3,24]]}},"alternative-id":["10.1126\/science.7892608"],"URL":"https:\/\/doi.org\/10.1126\/science.7892608","relation":{},"ISSN":["0036-8075","1095-9203"],"issn-type":[{"value":"0036-8075","type":"print"},{"value":"1095-9203","type":"electronic"}],"subject":[],"published":{"date-parts":[[1995,3,24]]}}}