{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T17:05:02Z","timestamp":1791219902743,"version":"4.1.0"},"reference-count":64,"publisher":"Rockefeller University Press","issue":"4","content-domain":{"domain":["rupress.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[1998,5,18]]},"abstract":"<jats:p>The characterization of scully, an essential gene of Drosophila with phenocritical phases at embryonic and pupal stages, shows its extensive homology with vertebrate type II l-3-hydroxyacyl-CoA dehydrogenase\/ERAB. Genomic rescue demonstrates that four different lethal mutations are scu alleles, the molecular nature of which has been established. One of them, scu3127, generates a nonfunctional truncated product. scu4058 also produces a truncated protein, but it contains most of the known functional domains of the enzyme. The other two mutations, scu174 and scuS152, correspond to single amino acid changes. The expression of scully mRNA is general to many tissues including the CNS; however, it is highest in both embryonic gonadal primordia and mature ovaries and testes. Consistent with this pattern, the phenotypic analysis suggests a role for scully in germ line formation: mutant testis are reduced in size and devoid of maturing sperm, and mutant ovarioles are not able to produce viable eggs. Ultrastructural analysis of mutant spermatocytes reveals the presence of cytoplasmic lipid inclusions and scarce mitochondria. In addition, mutant photoreceptors contain morphologically aberrant mitochondria and large multilayered accumulations of membranous material. Some of these phenotypes are very similar to those present in human pathologies caused by \u03b2-oxidation disorders.<\/jats:p>","DOI":"10.1083\/jcb.141.4.1009","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T16:47:50Z","timestamp":1027702070000},"page":"1009-1017","update-policy":"https:\/\/doi.org\/10.1083\/jcb.crossmarkpolicy","source":"Crossref","is-referenced-by-count":61,"title":["<i>scully<\/i>, an Essential Gene of <i>Drosophila<\/i>, is Homologous to Mammalian Mitochondrial Type II <scp>l<\/scp>-3-hydroxyacyl-CoA Dehydrogenase\/Amyloid-\u03b2 Peptide-binding Protein"],"prefix":"10.1083","volume":"141","author":[{"given":"Laura","family":"Torroja","sequence":"first","affiliation":[{"name":"Instituto Cajal, Consejo Superior de Investigaciones Cient\u00edficas, 28002 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Ortu\u00f1o-Sahag\u00fan","sequence":"additional","affiliation":[{"name":"Instituto Cajal, Consejo Superior de Investigaciones Cient\u00edficas, 28002 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Ferr\u00fas","sequence":"additional","affiliation":[{"name":"Instituto Cajal, Consejo Superior de Investigaciones Cient\u00edficas, 28002 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"H\u00e4mmerle","sequence":"additional","affiliation":[{"name":"Instituto Cajal, Consejo Superior de Investigaciones Cient\u00edficas, 28002 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julio A.","family":"Barbas","sequence":"additional","affiliation":[{"name":"Instituto Cajal, Consejo Superior de Investigaciones Cient\u00edficas, 28002 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[1998,5,18]]},"reference":[{"key":"2023072207332668100_B1","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1016\/S0022-2836(05)80360-2","article-title":"Basic local alignment search tool","volume":"215","author":"Altschul","year":"1990","journal-title":"J Mol Biol"},{"key":"2023072207332668100_B2","doi-asserted-by":"crossref","first-page":"14463","DOI":"10.1016\/S0021-9258(18)68242-0","article-title":"A noncleavable signal for mitochondrial import of 3-oxoacyl-CoA thiolase","volume":"263","author":"Amaya","year":"1988","journal-title":"J Biol Chem"},{"key":"2023072207332668100_B3","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1093\/oxfordjournals.jbchem.a123001","article-title":"The NH2-terminal 14\u201316 amino acids of mitochondrial and bacterial thiolases can direct mature ornithine carbamoyltransferase into mitochondria","volume":"107","author":"Arakawa","year":"1990","journal-title":"J Biochem"},{"key":"2023072207332668100_B4","doi-asserted-by":"crossref","first-page":"1359","DOI":"10.1093\/genetics\/139.3.1359","article-title":"Promoter elements in Drosophila melanogasterrevealed by sequence analysis","volume":"139","author":"Arkipova","year":"1995","journal-title":"Genetics"},{"key":"2023072207332668100_B5"},{"key":"2023072207332668100_B6","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1101\/gad.5.1.132","article-title":"Troponin I is encoded in the haplolethal region of the Shaker gene complex of Drosophila.","volume":"5","author":"Barbas","year":"1991","journal-title":"Genes Dev"},{"key":"2023072207332668100_B7","first-page":"1433","article-title":"Abnormal muscle development in the heldup3 mutant of Drosophila melanogaster is caused by a splicing defect affecting selected troponin I isoforms","volume":"13","author":"Barbas","year":"1993","journal-title":"Mol Cell Biol"},{"key":"2023072207332668100_B8","doi-asserted-by":"crossref","first-page":"3419","DOI":"10.1002\/j.1460-2075.1987.tb02665.x","article-title":"Molecular organization of the maternal effect region of the Shaker complex of Drosophila: characterization of an Ia channel transcript with homology to vertebrate Na+ channel","volume":"6","author":"Baumann","year":"1987","journal-title":"EMBO (Eur Mol Biol Organ) J"},{"key":"2023072207332668100_B9","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1016\/0166-2236(93)90117-5","article-title":"Do defects in mitochondrial energy metabolism underlie the pathology of neurodegenerative diseases?","volume":"16","author":"Beal","year":"1993","journal-title":"Trends Neurosci"},{"key":"2023072207332668100_B10","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1203\/00006450-199601000-00031","article-title":"Mitochondrial short-chain l-3-hydroxyacyl-coenzyme A dehydrogenase deficiency: a new defect of fatty acid oxidation","volume":"39","author":"Bennett","year":"1996","journal-title":"Pediatr Res"},{"key":"2023072207332668100_B11","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1042\/bj1340707","article-title":"The mitochondrial generation of hydrogen peroxide. General properties and effects on hyperbaric oxygen","volume":"134","author":"Boveris","year":"1973","journal-title":"Biochem J"},{"key":"2023072207332668100_B12","doi-asserted-by":"crossref","first-page":"3185","DOI":"10.1093\/nar\/19.12.3185","article-title":"Eukaryotic start and stop translation sites","volume":"19","author":"Cavener","year":"1991","journal-title":"Nucleic Acids Res"},{"key":"2023072207332668100_B13","first-page":"81","article-title":"The arthropod initiator: the cap site consensus plays an important role in transcription. Insect Biochem","volume":"23","author":"Cherbas","year":"1993","journal-title":"Mol Biol"},{"key":"2023072207332668100_B14","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1093\/genetics\/123.1.131","article-title":"Causes and consequences of variation in energy storage in Drosophila melanogaster.","volume":"123","author":"Clark","year":"1989","journal-title":"Genetics"},{"key":"2023072207332668100_B15","doi-asserted-by":"crossref","first-page":"679","DOI":"10.1126\/science.7901908","article-title":"Oxidative stress, glutamate, and neurodegenerative disorders","volume":"262","author":"Coyle","year":"1993","journal-title":"Science"},{"key":"2023072207332668100_B16","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1042\/bj3200345","article-title":"Mammalian mitochondrial beta-oxidation","volume":"320","author":"Eaton","year":"1996","journal-title":"Biochem J"},{"key":"2023072207332668100_B17","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1016\/0005-2760(82)90244-2","article-title":"The existence of an inner-membrane-bound, long acyl-chain-specific 3-hydroxyacyl-CoA dehydrogenase in mammalian mitochondria","volume":"713","author":"El-Fakhri","year":"1982","journal-title":"Biochim Biophys Acta"},{"key":"2023072207332668100_B18","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1016\/0003-2697(83)90418-9","article-title":"A technique for radiolabeling DNA restriction endonucleases fragments to high specific activity","volume":"132","author":"Feinberg","year":"1983","journal-title":"Anal Biochem"},{"key":"2023072207332668100_B19","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1093\/genetics\/125.2.383","article-title":"Genetic analysis of the Shakergene complex of Drosophila melanogaster","volume":"125","author":"Ferr\u00fas","year":"1990","journal-title":"Genetics"},{"key":"2023072207332668100_B20"},{"key":"2023072207332668100_B21","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1016\/S0167-4781(96)00171-6","article-title":"Cloning and expression of cDNA for a newly identified isozyme of bovine liver 3-hydroxyacyl-CoA dehydrogenase and its import into mitochondria","volume":"1350","author":"Furuta","year":"1997","journal-title":"Biochim Biophys Acta"},{"key":"2023072207332668100_B22","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1016\/0012-1606(72)90022-X","article-title":"Metabolic changes during spermatogenesis and thoracic tissue maturation in Drosophilahydei","volume":"28","author":"Geer","year":"1972","journal-title":"Dev Biol"},{"key":"2023072207332668100_B23","doi-asserted-by":"crossref","first-page":"10064","DOI":"10.1073\/pnas.88.22.10064","article-title":"Three-dimensional structure of holo 3 alpha, 20 beta-hydroxysteroid dehydrogenase: a member of a short-chain dehydrogenase family","volume":"88","author":"Ghosh","year":"1991","journal-title":"Proc Natl Acad Sci USA"},{"key":"2023072207332668100_B24","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1016\/S0969-2126(00)00064-2","article-title":"The refined three-dimensional structure of 3 alpha, 20 beta-hydroxy steroid dehydrogenase and possible roles of the residues conserved in short-chain dehydrogenases","volume":"2","author":"Ghosh","year":"1994","journal-title":"Structure"},{"key":"2023072207332668100_B25","doi-asserted-by":"crossref","first-page":"266","DOI":"10.1038\/ng0393-266","article-title":"Identification of protein coding regions by database similarity search","volume":"3","author":"Gish","year":"1993","journal-title":"Nat Genet"},{"key":"2023072207332668100_B26","doi-asserted-by":"crossref","first-page":"15334","DOI":"10.1016\/S0021-9258(19)49538-0","article-title":"Characterization and nucleotide binding properties of a mutant dihydropteridine reductase containing an aspartate 37-isoleucine replacement","volume":"267","author":"Grimshaw","year":"1992","journal-title":"J Biol Chem"},{"key":"2023072207332668100_B27","doi-asserted-by":"crossref","first-page":"C313","DOI":"10.1152\/ajpcell.1994.267.2.C313","article-title":"Mitochondrial calcium transport: physiological and pathological relevance","volume":"267","author":"Gunter","year":"1994","journal-title":"Am J Physiol"},{"key":"2023072207332668100_B28","doi-asserted-by":"crossref","first-page":"460","DOI":"10.1016\/0167-4781(93)90015-6","article-title":"Tissue specific and developmental expression of rat long- and medium-chain acyl-CoA dehydrogenases","volume":"1216","author":"Hainline","year":"1993","journal-title":"Biochim Biophys Acta"},{"key":"2023072207332668100_B29","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/0003-2697(89)90095-X","article-title":"Assay of l-3-hydroxyacyl-coenzyme A dehydrogenase with substrates of different chain lengths","volume":"180","author":"He","year":"1989","journal-title":"Anal Biochem"},{"key":"2023072207332668100_B30","doi-asserted-by":"crossref","first-page":"2038","DOI":"10.1074\/jbc.272.4.2038","article-title":"Nontoxic amyloid beta peptide 1\u201342 suppresses acetylcholine synthesis. Possible role in cholinergic dysfunction in Alzheimer's disease","volume":"272","author":"Hoshi","year":"1997","journal-title":"J Biol Chem"},{"key":"2023072207332668100_B31"},{"key":"2023072207332668100_B32"},{"key":"2023072207332668100_B33","doi-asserted-by":"crossref","first-page":"6003","DOI":"10.1021\/bi00018a001","article-title":"Short-chain dehydrogenases\/reductases (SDR)","volume":"34","author":"J\u00f6rnvall","year":"1995","journal-title":"Biochemistry"},{"key":"2023072207332668100_B34","doi-asserted-by":"crossref","first-page":"18921","DOI":"10.1016\/S0021-9258(19)47245-1","article-title":"The tissue-specific expression and developmental regulation of two nuclear genes encoding rat mitochondrial proteins. Medium chain acyl-CoA dehydrogenase and mitochondrial malate dehydrogenase","volume":"264","author":"Kelly","year":"1989","journal-title":"J Biol Chem"},{"key":"2023072207332668100_B35","doi-asserted-by":"crossref","first-page":"775","DOI":"10.1093\/oxfordjournals.jbchem.a021307","article-title":"Two mitochondrial 3-hydroxyacyl-CoA dehydrogenases in bovine liver","volume":"119","author":"Kobayashi","year":"1996","journal-title":"J Biochem"},{"key":"2023072207332668100_B36","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1016\/0960-0760(94)90084-1","article-title":"The short-chain alcohol dehydrogenase superfamily: variations on a common theme","volume":"51","author":"Krozowski","year":"1994","journal-title":"J Steroid Biochem Mol Biol"},{"key":"2023072207332668100_B37","doi-asserted-by":"crossref","first-page":"1172","DOI":"10.1093\/cvr\/26.12.1172","article-title":"Developmental changes in energy substrate use by the heart","volume":"26","author":"Lopaschuk","year":"1992","journal-title":"Cardiovasc Res"},{"key":"2023072207332668100_B38","doi-asserted-by":"crossref","first-page":"8731","DOI":"10.1073\/pnas.91.19.8731","article-title":"The development of mitochondrial medicine","volume":"91","author":"Luft","year":"1994","journal-title":"Proc Natl Acad Sci USA"},{"key":"2023072207332668100_B39","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1111\/j.1749-6632.1996.tb18611.x","article-title":"Functions and organization of peroxisomal beta-oxidation","volume":"804","author":"Mannaerts","year":"1996","journal-title":"Ann NY Acad Sci"},{"key":"2023072207332668100_B40","doi-asserted-by":"crossref","first-page":"4255","DOI":"10.1093\/nar\/20.16.4255","article-title":"Splicing signals in Drosophila: intron size, information content, and consensus sequences","volume":"20","author":"Mount","year":"1992","journal-title":"Nucleic Acids Res"},{"key":"2023072207332668100_B41","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1021\/bi00351a019","article-title":"Human placental estradiol 17 beta-dehydrogenase: sequence of a histidine-bearing peptide in the catalytic region","volume":"25","author":"Murdock","year":"1986","journal-title":"Biochemistry"},{"key":"2023072207332668100_B42","doi-asserted-by":"crossref","first-page":"2218","DOI":"10.1074\/jbc.272.4.2218","article-title":"Switch of coenzyme specificity of mouse lung carbonyl reductase by substitution of threonine 38 with aspartic acid","volume":"272","author":"Nakanishi","year":"1997","journal-title":"J Biol Chem"},{"key":"2023072207332668100_B43","doi-asserted-by":"crossref","first-page":"1027","DOI":"10.1016\/0092-8674(84)90437-9","article-title":"The Drosophila ras oncogenes: structure and nucleotide sequence","volume":"37","author":"Neuman-Silberberg","year":"1984","journal-title":"Cell"},{"key":"2023072207332668100_B44","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1016\/0005-2760(79)90007-9","article-title":"Occurrence of two 3-hydroxyacyl-CoA dehydrogenases in rat liver","volume":"574","author":"Osumi","year":"1979","journal-title":"Biochim Biophys Acta"},{"key":"2023072207332668100_B45","doi-asserted-by":"crossref","first-page":"2444","DOI":"10.1073\/pnas.85.8.2444","article-title":"Improved tools for biological sequence comparison","volume":"85","author":"Pearson","year":"1988","journal-title":"Proc Natl Acad Sci USA"},{"key":"2023072207332668100_B46","first-page":"437","article-title":"Vectors for P-mediated transformation in Drosophila","volume":"10","author":"Pirrotta","year":"1988","journal-title":"Biotechnology"},{"key":"2023072207332668100_B47","doi-asserted-by":"crossref","first-page":"236","DOI":"10.1016\/0887-8994(96)00021-5","article-title":"The clinical spectrum of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency","volume":"14","author":"Pons","year":"1996","journal-title":"Pediatr Neurol"},{"key":"2023072207332668100_B48","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1111\/j.1471-4159.1988.tb01044.x","article-title":"Enzymes of fatty acid beta-oxidation in developing brain","volume":"51","author":"Reichmann","year":"1988","journal-title":"J Neurochem"},{"key":"2023072207332668100_B49","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1095\/biolreprod42.3.511","article-title":"Support of Xenopus laevis spermatogenesis in vitro by different energy substrates","volume":"42","author":"Risley","year":"1990","journal-title":"Biol Reprod"},{"key":"2023072207332668100_B50"},{"key":"2023072207332668100_B51"},{"key":"2023072207332668100_B52"},{"key":"2023072207332668100_B53","doi-asserted-by":"crossref","first-page":"5463","DOI":"10.1073\/pnas.74.12.5463","article-title":"DNA sequencing with chain terminating inhibitors","volume":"74","author":"Sanger","year":"1977","journal-title":"Proc Natl Acad Sci USA"},{"key":"2023072207332668100_B54","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1007\/BF00267823","article-title":"A single protocol to detect transcripts of various types and expression levels in neural tissue and cultured cells: in situ hybridization using digoxigenin-labeled cRNA probes","volume":"100","author":"Schaeren-Wiemers","year":"1993","journal-title":"Histochemistry"},{"key":"2023072207332668100_B55","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1002\/ana.410400409","article-title":"Trifunctional enzyme deficiency: adult presentation of a usually fatal beta-oxidation defect","volume":"40","author":"Schaefer","year":"1996","journal-title":"Ann Neurol"},{"key":"2023072207332668100_B56","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1006\/neur.1996.0059","article-title":"Energy metabolism, oxidative stress and neuronal degeneration in Alzheimer's disease","volume":"5","author":"Sims","year":"1996","journal-title":"Neurodegeneration"},{"key":"2023072207332668100_B57","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1126\/science.6289435","article-title":"Transposition of cloned P elements into Drosophila germ line chromosomes","volume":"218","author":"Spradling","year":"1982","journal-title":"Science"},{"key":"2023072207332668100_B58","doi-asserted-by":"crossref","first-page":"7715","DOI":"10.1021\/bi951904d","article-title":"Crystal structures of the binary and ternary complexes of 7 alpha- hydroxysteroid dehydrogenase from Escherichia coli","volume":"35","author":"Tanaka","year":"1996","journal-title":"Biochemistry"},{"key":"2023072207332668100_B59","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1002\/ana.410300315","article-title":"Short-chain l-3-hydroxyacyl-CoA dehydrogenase deficiency in muscle: a new cause for recurrent myoglobinuria and encephalopathy","volume":"30","author":"Tein","year":"1991","journal-title":"Ann Neurol"},{"key":"2023072207332668100_B60","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1002\/ana.410270214","article-title":"Metabolic causes of myoglobinuria","volume":"27","author":"Tonin","year":"1990","journal-title":"Ann Neurol"},{"key":"2023072207332668100_B61","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1080\/15513819709168585","article-title":"Pathology of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency caused by the G1528C mutation","volume":"17","author":"Tyni","year":"1997","journal-title":"Pediatr Pathol Lab Med"},{"key":"2023072207332668100_B62","doi-asserted-by":"crossref","first-page":"6080","DOI":"10.1073\/pnas.89.13.6080","article-title":"Crystal structure of rat liver dihydropteridine reductase","volume":"89","author":"Varughese","year":"1992","journal-title":"Proc Natl Acad Sci USA"},{"key":"2023072207332668100_B63","doi-asserted-by":"crossref","first-page":"1045","DOI":"10.1126\/science.6208611","article-title":"Role of the conserved AAUAAA sequence: four AAUAAA point mutants prevent messenger RNA 3\u2032 end formation","volume":"226","author":"Wickens","year":"1984","journal-title":"Science"},{"key":"2023072207332668100_B64","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1038\/39522","article-title":"An intracellular protein that binds amyloid-\u03b2 peptide and mediates neurotoxicity in Alzheimer's disease","volume":"389","author":"Yan","year":"1997","journal-title":"Nature"}],"container-title":["The Journal of Cell Biology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/rupress.org\/jcb\/article-pdf\/141\/4\/1009\/1490275\/9801039.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/rupress.org\/jcb\/article-pdf\/141\/4\/1009\/1490275\/9801039.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,7,22]],"date-time":"2023-07-22T07:34:42Z","timestamp":1690011282000},"score":1,"resource":{"primary":{"URL":"https:\/\/rupress.org\/jcb\/article\/141\/4\/1009\/15781\/scully-an-Essential-Gene-of-Drosophila-is"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1998,5,18]]},"references-count":64,"journal-issue":{"issue":"4","published-print":{"date-parts":[[1998,5,18]]}},"URL":"https:\/\/doi.org\/10.1083\/jcb.141.4.1009","relation":{},"ISSN":["0021-9525","1540-8140"],"issn-type":[{"value":"0021-9525","type":"print"},{"value":"1540-8140","type":"electronic"}],"subject":[],"published":{"date-parts":[[1998,5,18]]}}}