Acads

Summary

Gene Symbol: Acads
Description: acyl-Coenzyme A dehydrogenase, short chain
Alias: AI196007, Bcd-1, Bcd1, Hdlq8, SCAD, short-chain specific acyl-CoA dehydrogenase, mitochondrial, butyryl-CoA dehydrogenase
Species: mouse
Products:     Acads

Top Publications

  1. Kelly C, Wood P. Cloning and characterization of the mouse short-chain acyl-CoA dehydrogenase gene. Mamm Genome. 1996;7:262-4 pubmed
    ..there is coordinate regulation between these similar genes, we have isolated genomic clones containing the mouse Acads gene. We show that Acads is a compact, single-copy gene approximately 5000 bp in size...
  2. Wood P, Kelly Kurtz C, Hinsdale M, Hamm D, Rhead W. Lessons learned from the mouse model of short-chain acyl-CoA dehydrogenase deficiency. Adv Exp Med Biol. 1999;466:395-402 pubmed
    The SCAD deficient mouse model has been useful to investigate mechanisms of deficient fatty acid oxidation disease in human patients. This mouse model has been thoroughly characterized and is readily available from the Jackson Laboratory...
  3. Schiffer S, Prochazka M, Jezyk P, Roderick T, Yudkoff M, Patterson D. Organic aciduria and butyryl CoA dehydrogenase deficiency in BALB/cByJ mice. Biochem Genet. 1989;27:47-58 pubmed
    ..Independently from this screening a variant with no detectable enzyme activity of butyryl CoA dehydrogenase (BCD) in liver and kidney of the BALB/cByJ strain but not other BALB/c sublines was discovered...
  4. Smith Richards B, Belton B, York B, Volaufova J. Mice bearing Acads mutation display altered postingestive but not 5-s orosensory response to dietary fat. Am J Physiol Regul Integr Comp Physiol. 2004;286:R311-9 pubmed
    ..mice are deficient in the short-chain acyl-CoA dehydrogenase (SCAD) enzyme due to a spontaneous mutation in Acads. We hypothesized that this deficiency would alter fat appetite and used three behavioral test paradigms to compare ..
  5. Amendt B, Freneaux E, Reece C, Wood P, Rhead W. Short-chain acyl-coenzyme A dehydrogenase activity, antigen, and biosynthesis are absent in the BALB/cByJ mouse. Pediatr Res. 1992;31:552-6 pubmed
    BALB/cByJ (J) mice have short-chain acyl-CoA dehydrogenase (SCAD) deficiency and an organic aciduria similar to that of human SCAD deficiency...
  6. Wood P, Amendt B, Rhead W, Millington D, Inoue F, Armstrong D. Short-chain acyl-coenzyme A dehydrogenase deficiency in mice. Pediatr Res. 1989;25:38-43 pubmed
    A murine model for short-chain acyl-coenzyme A dehydrogenase (SCAD) deficiency has been identified and characterized in BALB/cByJ mice...
  7. Reue K, Cohen R. Acads gene deletion in BALB/cByJ mouse strain occurred after 1981 and is not present in BALB/cByJ-fld mutant mice. Mamm Genome. 1996;7:694-5 pubmed
  8. Hinsdale M, Kelly C, Wood P. Null allele at Bcd-1 locus in BALB/cByJ mice is due to a deletion in the short-chain acyl-CoA dehydrogenase gene and results in missplicing of mRNA. Genomics. 1993;16:605-11 pubmed
    BALB/cByJ mice have a deficiency of short-chain acyl-CoA dehydrogenase (SCAD), an enzyme of fatty acid beta-oxidation. This mutant mouse strain represents the only animal model for any human inborn error of fatty acid metabolism...
  9. Su Z, Ishimori N, Chen Y, Leiter E, Churchill G, Paigen B, et al. Four additional mouse crosses improve the lipid QTL landscape and identify Lipg as a QTL gene. J Lipid Res. 2009;50:2083-94 pubmed publisher
    ..The data from these crosses further increase the ability to perform haplotype analyses that can lead to the identification of causal lipid genes. ..

More Information

Publications40

  1. Hinsdale M, Hamm D, Wood P. Effects of short-chain acyl-CoA dehydrogenase deficiency on development expression of metabolic enzyme genes in the mouse. Biochem Mol Med. 1996;57:106-15 pubmed
    ..throughout neonatal development, the steady-state mRNA levels of long-chain, medium-chain, and short-chain (SCAD) acyl-CoA dehydrogenases, pyruvate carboxylase (PC), phosphoenolpyruvate carboxykinase (PEPCK), carbamyl phosphate ..
  2. Armstrong D, Masiowski M, Wood P. Pathologic characterization of short-chain acyl-CoA dehydrogenase deficiency in BALB/cByJ mice. Am J Med Genet. 1993;47:884-92 pubmed
    BALB/cByJ mice have a deficiency of short-chain acyl-CoA dehydrogenase (SCAD) and are a useful model for studying the inborn errors of fatty acid metabolism which affect humans...
  3. Patarca R, Freeman G, Singh R, Wei F, Durfee T, Blattner F, et al. Structural and functional studies of the early T lymphocyte activation 1 (Eta-1) gene. Definition of a novel T cell-dependent response associated with genetic resistance to bacterial infection. J Exp Med. 1989;170:145-61 pubmed
    ..Eta-1 gene expression appears to be part of a surprisingly rapid T cell-dependent response to bacterial infection that may precede classical forms of T cell-dependent immunity. ..
  4. Skilling H, Coen P, Fairfull L, Ferrell R, Goodpaster B, Vockley J, et al. Brown adipose tissue function in short-chain acyl-CoA dehydrogenase deficient mice. Biochem Biophys Res Commun. 2010;400:318-22 pubmed publisher
    ..Short-chain acyl-CoA dehydrogenase (SCAD) deficient mice were found to have increased brown adipose tissue mass as well as modest cardiac hypertrophy...
  5. Kruger C, Kumar K, Mynatt R, Volaufova J, Richards B. Brain transcriptional responses to high-fat diet in Acads-deficient mice reveal energy sensing pathways. PLoS ONE. 2012;7:e41709 pubmed publisher
    ..Previously we reported that mice with a genetic inactivation of Acads (acyl-coenzyme A dehydrogenase, short-chain), the enzyme responsible for mitochondrial beta-oxidation of C4-C6 ..
  6. Bussoli T, Kelly A, Steel K. Localization of the bronx waltzer (bv) deafness gene to mouse chromosome 5. Mamm Genome. 1997;8:714-7 pubmed
    ..bv maps 0.14 cM distal of the marker D5Mit209 and 1.14 cM proximal of the marker D5Mit188 in 701 backcross progeny. ..
  7. Maichele A, Chamberlain J. The gamma phosphorylase kinase gene, Phkg, maps to mouse chromosome 5 near Gus. Mamm Genome. 1994;5:15-8 pubmed
  8. Wang X, Le Roy I, Nicodeme E, Li R, Wagner R, Petros C, et al. Using advanced intercross lines for high-resolution mapping of HDL cholesterol quantitative trait loci. Genome Res. 2003;13:1654-64 pubmed
    ..All the major HDL QTLs in our study had homologous counterparts in humans, implying that their underlying genes regulate HDL in humans. ..
  9. Holm D, Dagnaes Hansen F, Simonsen H, Gregersen N, Bolund L, Jensen T, et al. Expression of short-chain acyl-CoA dehydrogenase (SCAD) proteins in the liver of SCAD deficient mice after hydrodynamic gene transfer. Mol Genet Metab. 2003;78:250-8 pubmed
    ..in vivo expression of variant proteins involved in metabolic diseases, using short-chain acyl-CoA dehydrogenase (SCAD) deficient mice (BALB/cByJ) as a model...
  10. Wang W, Mohsen A, Uechi G, Schreiber E, Balasubramani M, DAY B, et al. Complex changes in the liver mitochondrial proteome of short chain acyl-CoA dehydrogenase deficient mice. Mol Genet Metab. 2014;112:30-9 pubmed publisher
    Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is an autosomal recessive inborn error of metabolism that leads to the impaired mitochondrial fatty acid ?-oxidation of short chain fatty acids...
  11. Beattie S, Goetzman E, Conlon T, Germain S, Walter G, Campbell Thompson M, et al. Biochemical correction of short-chain acyl-coenzyme A dehydrogenase deficiency after portal vein injection of rAAV8-SCAD. Hum Gene Ther. 2008;19:579-88 pubmed publisher
    ..would produce sufficient hepatocyte transduction (after administration via the portal vein) and thus sufficient SCAD enzyme to correct the phenotype observed in the SCAD-deficient (BALB/cByJ) mouse, which includes elevated blood ..
  12. Seeley T, Holmes R. Genetics and ontogeny of butyryl CoA dehydrogenase in the mouse and linkage of Bcd-1 with Dao-1. Biochem Genet. 1981;19:333-45 pubmed
    ..been developed for fatty acyl CoA dehydrogenase and used to examine the electrophoretic properties of butyryl CoA dehydrogenase (BCD) from mouse tissues...
  13. Andresen B, Corydon T, Wilsbech M, Bross P, Schroeder L, Hindkjaer T, et al. Characterization of mouse Clpp protease cDNA, gene, and protein. Mamm Genome. 2000;11:275-80 pubmed
  14. Kurtz D, Rinaldo P, Rhead W, Tian L, Millington D, Vockley J, et al. Targeted disruption of mouse long-chain acyl-CoA dehydrogenase gene reveals crucial roles for fatty acid oxidation. Proc Natl Acad Sci U S A. 1998;95:15592-7 pubmed
    ..Approximately 10% of adult LCAD -/- males developed cardiomyopathy, and sudden death was observed in 4 of 75 LCAD -/- mice. These results demonstrate the crucial roles of mitochondrial FAO and LCAD in vivo. ..
  15. Giesen K, Plum L, Kluge R, Ortlepp J, Joost H. Diet-dependent obesity and hypercholesterolemia in the New Zealand obese mouse: identification of a quantitative trait locus for elevated serum cholesterol on the distal mouse chromosome 5. Biochem Biophys Res Commun. 2003;304:812-7 pubmed
    ..The effect of Chol1/NZO was independent of the dietary fat content and was not associated with the other traits of the metabolic syndrome. Thus, it is suggested that the responsible gene might be involved in cholesterol metabolism. ..
  16. Pedersen C, Bross P, Winter V, Corydon T, Bolund L, Bartlett K, et al. Misfolding, degradation, and aggregation of variant proteins. The molecular pathogenesis of short chain acyl-CoA dehydrogenase (SCAD) deficiency. J Biol Chem. 2003;278:47449-58 pubmed
    Short chain acyl-CoA dehydrogenase (SCAD) deficiency is an inborn error of the mitochondrial fatty acid metabolism caused by rare variations as well as common susceptibility variations in the SCAD gene...
  17. Winchester G, Mitchison N, Taylor B. The structural gene for F liver protein (Flp) maps to chromosome 5 of the mouse. Immunogenetics. 1987;26:356-8 pubmed
    ..The typed RI strains have then been used for the immunogenetic studies presented in the succeeding article. ..
  18. Su Z, Leduc M, Korstanje R, Paigen B. Untangling HDL quantitative trait loci on mouse chromosome 5 and identifying Scarb1 and Acads as the underlying genes. J Lipid Res. 2010;51:2706-13 pubmed publisher
    Two high-density lipoprotein cholesterol quantitative trait loci (QTL), Hdlq1 at 125 Mb and Hdlq8 at 113 Mb, were previously identified on mouse distal chromosome 5. Our objective was to identify the underlying genes...
  19. Kelly C, Hinsdale M, Wood P. Cloning and characterization of the mouse short-chain acyl-CoA dehydrogenase cDNA. Genomics. 1993;18:137-40 pubmed
    Short-chain acyl-CoA dehydrogenase (SCAD) is one of five homologous dehydrogenases that catalyze the first reaction in the beta-oxidation of fatty acids. As the name implies, the substrate for this enzyme is short-chain acyl-CoA (C4-C6)...
  20. Pontoglio M, Barra J, Hadchouel M, Doyen A, Kress C, Bach J, et al. Hepatocyte nuclear factor 1 inactivation results in hepatic dysfunction, phenylketonuria, and renal Fanconi syndrome. Cell. 1996;84:575-85 pubmed
    ..The resulting massive urinary glucose loss leads to energy and water wasting. HNF1-deficient mice may provide a model for human renal Fanconi syndrome...
  21. Ramírez Torres A, Barceló Batllori S, Fernandez Vizarra E, Navarro M, Arnal C, Guillen N, et al. Proteomics and gene expression analyses of mitochondria from squalene-treated apoE-deficient mice identify short-chain specific acyl-CoA dehydrogenase changes associated with fatty liver amelioration. J Proteomics. 2012;75:2563-75 pubmed publisher
    ..Methionine adenosyltransferase I alpha (Mat1a) and short-chain specific acyl-CoA dehydrogenase (Acads) showed significant increased and decreased transcripts, respectively, consistent with their protein changes...
  22. Kaiko G, Ryu S, Koues O, Collins P, Solnica Krezel L, Pearce E, et al. The Colonic Crypt Protects Stem Cells from Microbiota-Derived Metabolites. Cell. 2016;165:1708-1720 pubmed publisher
  23. Stylianou I, Affourtit J, Shockley K, Wilpan R, Abdi F, Bhardwaj S, et al. Applying gene expression, proteomics and single-nucleotide polymorphism analysis for complex trait gene identification. Genetics. 2008;178:1795-805 pubmed publisher
    ..For example, candidate genes such as Apoa2 and Acads had differential protein levels although the mRNA levels were similar...
  24. Schuler A, Gower B, Matern D, Rinaldo P, Vockley J, Wood P. Synergistic heterozygosity in mice with inherited enzyme deficiencies of mitochondrial fatty acid beta-oxidation. Mol Genet Metab. 2005;85:7-11 pubmed
    ..dehydrogenase, long-chain acyl CoA dehydrogenase, and short-chain acyl CoA dehydrogenase genes (VLCAD+/-//LCAD+/-//SCAD+/-); double heterozygosity for mutations in VLCAD and LCAD genes (VLCAD+/-//LCAD+/-); double heterozygosity for ..
  25. Schuler A, Gower B, Matern D, Rinaldo P, Wood P. Influence of dietary fatty acid chain-length on metabolic tolerance in mouse models of inherited defects in mitochondrial fatty acid beta-oxidation. Mol Genet Metab. 2004;83:322-9 pubmed
    ..challenge, we fed long-chain acyl CoA dehydrogenase (LCAD) deficient and short-chain acyl CoA dehydrogenase (SCAD) deficient mice a diet rich in medium-chain triglycerides (MCT) or long-chain triglycerides (LCT)...
  26. Gragnoli C, Lindner T, Cockburn B, Kaisaki P, Gragnoli F, Marozzi G, et al. Maturity-onset diabetes of the young due to a mutation in the hepatocyte nuclear factor-4 alpha binding site in the promoter of the hepatocyte nuclear factor-1 alpha gene. Diabetes. 1997;46:1648-51 pubmed
    ..Moreover, it indicates that both the promoter and coding regions of the HNF-1 alpha gene should be screened for mutations in subjects thought to have MODY because of mutations in this gene. ..
  27. Conlon T, Walter G, Owen R, Cossette T, Erger K, Gutierrez G, et al. Systemic correction of a fatty acid oxidation defect by intramuscular injection of a recombinant adeno-associated virus vector. Hum Gene Ther. 2006;17:71-80 pubmed
    ..We have used recombinant adeno-associated virus (rAAV) vectors expressing short-chain acyl-CoA dehydrogenase (SCAD) to correct the accumulation of fatty acyl-CoA intermediates in deficient cell lines...
  28. Guerra C, Koza R, Walsh K, Kurtz D, Wood P, Kozak L. Abnormal nonshivering thermogenesis in mice with inherited defects of fatty acid oxidation. J Clin Invest. 1998;102:1724-31 pubmed
    ..Genetic mapping of the trait placed the gene on chromosome 5 near Acads, a gene encoding the short chain acyl CoA dehydrogenase, which is mutated in BALB/cByJ mice...
  29. Palladino A, Chen J, Kallish S, Stanley C, Bennett M. Measurement of tissue acyl-CoAs using flow-injection tandem mass spectrometry: acyl-CoA profiles in short-chain fatty acid oxidation defects. Mol Genet Metab. 2012;107:679-83 pubmed publisher
    ..We validated the method using liver of the short-chain-acyl-CoA dehydrogenase (SCAD) knock-out mice...
  30. Qureshi I, LeBlanc D, Cyr D, Giguere R, Mitchell G. Breeding experiments to combine the X-linked sparse-fur (spf) mutation with the autosomal recessive BALB/cByJ strain: testing the biochemical phenotype of double-mutant mice as a model for ammonia: fatty acyl CoA synergism. Biochem Biophys Res Commun. 1993;191:744-9 pubmed
    ..ornithine transcarbamylase deficiency and the autosomal recessive deficiency of short-chain acyl CoA dehydrogenase (SCAD) in BALB/cByJ mice...
  31. Kuo C, Conley P, Hsieh C, Francke U, Crabtree G. Molecular cloning, functional expression, and chromosomal localization of mouse hepatocyte nuclear factor 1. Proc Natl Acad Sci U S A. 1990;87:9838-42 pubmed