{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T13:40:35Z","timestamp":1777470035945,"version":"3.51.4"},"reference-count":19,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2005,6,3]],"date-time":"2005-06-03T00:00:00Z","timestamp":1117756800000},"content-version":"vor","delay-in-days":4232,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Am. J. Med. Genet."],"published-print":{"date-parts":[[1993,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>BALB\/cByJ mice have a deficiency of short\u2010chain acyl\u2010CoA dehydrogenase (SCAD) and are a useful model for studying the inborn errors of fatty acid metabolism which affect humans. Patients with some of these disorders present with hypoglycemia, hyperamonemia, and microvesicular fatty change of hepatocytes. In the present study we examined pathogen\u2010free, SCAD deficient BALB\/cByJ mice and control BALB\/cBy mice for biochemical and tissue changes following fasting or salicylate challenge. We observed mitochondrial swelling and microvesicular fatty changes in hepatocytes in mutant mice, especially severe following a fast. However, fasting did not alter their blood ammonia and there was no apparent clinical disease. Similarly, salicylates did not produce disease in the BALB\/cByJ mice. We did detect in mice an alternative pathway for salicylate metabolism, by\u2010passing glycine conjugation which is the principal metabolic pathway in humans. \u00a9 1993 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.1320470616","type":"journal-article","created":{"date-parts":[[2005,6,11]],"date-time":"2005-06-11T16:41:37Z","timestamp":1118508097000},"page":"884-892","source":"Crossref","is-referenced-by-count":20,"title":["Pathologic characterization of short\u2010chain acyl\u2010CoA dehydrogenase deficiency in BALB\/cByJ mice"],"prefix":"10.1002","volume":"47","author":[{"given":"Dawna L.","family":"Armstrong","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael L.","family":"Masiowski","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philip A.","family":"Wood","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,6,3]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(85)80237-7"},{"key":"e_1_2_1_3_1","first-page":"386","article-title":"Spontaneous Reye's\u2010like syndrome in BALB\/cByJ mice","volume":"51","author":"Brownstein DG","year":"1984","journal-title":"Lab Invest"},{"key":"e_1_2_1_4_1","first-page":"127","article-title":"Reye syndrome: Light and electron microscopic studies","volume":"96","author":"Chang LW","year":"1973","journal-title":"Arch Pathol Lab Med"},{"key":"e_1_2_1_5_1","first-page":"313","article-title":"A morphometric study of Reye's syndrome: Correlation of reduced mitochondrial numbers and increased mitochondrial size with clinical manifestations","volume":"129","author":"Daugherty CC","year":"1987","journal-title":"Am J Pathol"},{"key":"e_1_2_1_6_1","first-page":"19","volume-title":"Muscle Biopsy: A Modern Approach","author":"Dobowitz V","year":"1973"},{"key":"e_1_2_1_7_1","first-page":"694","volume-title":"The Pharmacological Basis of Therapeutics","author":"Goodman AGG","year":"1980"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(88)80551-1"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.epirev.a036043"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0046-8177(77)80006-3"},{"key":"e_1_2_1_11_1","first-page":"325","volume-title":"The Urea Cycle","author":"Nuzum CT","year":"1976"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197112092852402"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-197509000-00005"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-197811000-00007"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(63)90554-3"},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(86)80762-4"},{"key":"e_1_2_1_17_1","first-page":"489","volume-title":"Progress in Liver Disease","author":"Schubert WK","year":"1972"},{"key":"e_1_2_1_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840060608"},{"key":"e_1_2_1_19_1","first-page":"15","article-title":"Screening mutant mice for inborn errors of metabolism","volume":"38","author":"Wood PA","year":"1988","journal-title":"Lab Anim Sci"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-198901000-00010"}],"container-title":["American Journal of Medical Genetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajmg.1320470616","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajmg.1320470616","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,25]],"date-time":"2023-10-25T07:17:59Z","timestamp":1698218279000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajmg.1320470616"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1993,11]]},"references-count":19,"journal-issue":{"issue":"6","published-print":{"date-parts":[[1993,11]]}},"alternative-id":["10.1002\/ajmg.1320470616"],"URL":"https:\/\/doi.org\/10.1002\/ajmg.1320470616","archive":["Portico"],"relation":{},"ISSN":["0148-7299","1096-8628"],"issn-type":[{"value":"0148-7299","type":"print"},{"value":"1096-8628","type":"electronic"}],"subject":[],"published":{"date-parts":[[1993,11]]}}}