acyl-CoA dehydrogenase short chain ; Homo sapiens

60 Article(s)
Pub. Date
#Total Relationship(s)
1 1692038 Identification of two variant short chain acyl-coenzyme A dehydrogenase alleles, each containing a different point mutation in a patient with short chain acyl-coenzyme A dehydrogenase deficiency. J Clin Invest 1990 May 4
2 2340266 Spectral and electrochemical properties of glutaryl-CoA dehydrogenase from Paracoccus denitrificans. Biochemistry 1990 Apr 17 2
3 2774489 Normalization of short-chain acylcoenzyme A dehydrogenase after riboflavin treatment in a girl with multiple acylcoenzyme A dehydrogenase-deficient myopathy. Ann Neurol 1989 May 2
4 2808706 Short chain acyl-coenzyme A dehydrogenase (SCAD) deficiency. Immunochemical demonstration of molecular heterogeneity due to variant SCAD with differing stability. J Clin Invest 1989 Nov 14
5 3607039 Regulation of the butyryl-CoA dehydrogenase by substrate and product binding. Biochemistry 1987 May 5 1
6 4797895 Biochemical effects of the hypoglycaemic compound pent-4-enoic acid and related non-hypoglycaemic fatty acids. Effects of their coenzyme A esters on enzymes of fatty acid oxidation. Biochem J 1973 Sep 3
7 6882359 The effect on butyryl-CoA dehydrogenase of reagents specific for nucleophilic sulphur. Biochem J 1983 Jun 1 2
8 7068637 Evidence that the greening ligand in native butyryl-CoA dehydrogenase is a CoA persulfide. J Biol Chem 1982 Apr 25 2
9 7126602 A convenient and rapid method for the complete removal of CoA from butyryl-CoA dehydrogenase. Biochim Biophys Acta 1982 Sep 7 2
10 7208150 Glutaric aciduria type II: in vitro studies on substrate oxidation, acyl-CoA dehydrogenases, and electron-transferring flavoprotein in cultured skin fibroblasts. Pediatr Res 1980 Dec 2
11 7988059 Measurement of short-chain acyl-CoA dehydrogenase (SCAD) in cultured skin fibroblasts with hexanoyl-CoA as a competitive inhibitor to eliminate the contribution of medium-chain acyl-CoA dehydrogenase. Clin Chim Acta 1994 Sep 3
12 8102510 A rare disease-associated mutation in the medium-chain acyl-CoA dehydrogenase (MCAD) gene changes a conserved arginine, previously shown to be functionally essential in short-chain acyl-CoA dehydrogenase (SCAD). Am J Hum Genet 1993 Sep 4
13 8399220 Characterization of wild-type and an active-site mutant in Escherichia coli of short-chain acyl-CoA dehydrogenase from Megasphaera elsdenii. Biochemistry 1993 Oct 12 2
14 8725270 Ethylmalonic aciduria is associated with an amino acid variant of short chain acyl-coenzyme A dehydrogenase. Pediatr Res 1996 Jun 10
15 9383286 Structural organization of the human short-chain acyl-CoA dehydrogenase gene. Mamm Genome 1997 Dec 4
16 9499414 Identification of four new mutations in the short-chain acyl-CoA dehydrogenase (SCAD) gene in two patients: one of the variant alleles, 511C-->T, is present at an unexpectedly high frequency in the general population, as was the case for 625G-->A, together conferring susceptibility to ethylmalonic aciduria. Hum Mol Genet 1998 Apr 3
17 10198089 Identification, separation, and characterization of acyl-coenzyme A dehydrogenases involved in mitochondrial beta-oxidation in higher plants Plant Physiol 1999 Apr 2
18 11013134 Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency: identification of a new enzyme defect, resolution of its molecular basis, and evidence for distinct acyl-CoA dehydrogenases in isoleucine and valine metabolism. Am J Hum Genet 2000 Nov 2
19 11216903 Prevalent mutations in fatty acid oxidation disorders: diagnostic considerations. Eur J Pediatr 2000 Dec 2
20 11231285 Purification, characterization and cloning of isovaleryl-CoA dehydrogenase from higher plant mitochondria. Eur J Biochem 2001 Mar 1
21 11524729 Mutation analysis in mitochondrial fatty acid oxidation defects: Exemplified by acyl-CoA dehydrogenase deficiencies, with special focus on genotype-phenotype relationship. Hum Mutat 2001 Sep 3
22 12061367 Hypoglycaemia and elevated urine ethylmalonic acid in a child homozygous for the short-chain acyl-CoA dehydrogenase 625G > A gene variation. Acta Paediatr 2002 5
23 12706374 The frequency of short-chain acyl-CoA dehydrogenase gene variants in the US population and correlation with the C(4)-acylcarnitine concentration in newborn blood spots. Mol Genet Metab 2003 Apr 2
24 12736383 Rare disorders of metabolism with elevated butyryl- and isobutyryl-carnitine detected by tandem mass spectrometry newborn screening. Pediatr Res 2003 Aug 1
25 14568186 A comparison of in vitro acylcarnitine profiling methods for the diagnosis of classical and variant short chain acyl-CoA dehydrogenase deficiency. Clin Chim Acta 2003 Nov 5
26 14595061 Short-chain Acyl-CoA dehydrogenase deficiency: studies in a large family adding to the complexity of the disorder. Pediatrics 2003 Nov 2
27 16020546 Human acyl-CoA dehydrogenase-9 plays a novel role in the mitochondrial beta-oxidation of unsaturated fatty acids. J Biol Chem 2005 Sep 16 2
28 16101709 Perioperative management of a child with short-chain acyl-CoA dehydrogenase deficiency. Paediatr Anaesth 2005 Sep 4
29 16331963 Biochemical and electrochemical characterization of two variant human short-chain acyl-CoA dehydrogenases. Biochemistry 2005 Dec 13 1
30 16331964 Thermodynamic regulation of human short-chain acyl-CoA dehydrogenase by substrate and product binding. Biochemistry 2005 Dec 13 1
31 16376132 Functional analysis of acyl-CoA dehydrogenase catalytic residue mutants using surface plasmon resonance and circular dichroism. Mol Genet Metab 2006 Mar 1
32 18523805 The ACADS gene variation spectrum in 114 patients with short-chain acyl-CoA dehydrogenase (SCAD) deficiency is dominated by missense variations leading to protein misfolding at the cellular level. Hum Genet 2008 Aug 1
33 19625492 Diversity and dispersal of a ubiquitous protein family: acyl-CoA dehydrogenases. Nucleic Acids Res 2009 Sep 3
34 19639238 Acyl-CoA dehydrogenases: Dynamic history of protein family evolution. J Mol Evol 2009 Aug 4
35 20376488 Molecular pathogenesis of a novel mutation, G108D, in short-chain acyl-CoA dehydrogenase identified in subjects with short-chain acyl-CoA dehydrogenase deficiency. Hum Genet 2010 Jun 2
36 20443061 Antioxidant dysfunction: potential risk for neurotoxicity in ethylmalonic aciduria. J Inherit Metab Dis 2010 Jun 1
37 21211036 The minor C-allele of rs2014355 in ACADS is associated with reduced insulin release following an oral glucose load. BMC Med Genet 2011 Jan 6 4
38 21237683 Identification and characterization of new long chain acyl-CoA dehydrogenases. Mol Genet Metab 2011 Apr 4
39 21325261 Mutations of ACADS gene associated with short-chain acyl-coenzyme A dehydrogenase deficiency. Ann Clin Lab Sci 2011 Fall 1
40 21987487 Impaired butyrate oxidation in ulcerative colitis is due to decreased butyrate uptake and a defect in the oxidation pathway. Inflamm Bowel Dis 2012 Jun 1
41 24478438 Forkhead box transcription factor regulation and lipid accumulation by hepatitis C virus. J Virol 2014 Apr 2
42 26031853 Role of peroxisome proliferator-activated receptor alpha in the expression of hepatic fatty acid oxidation-related genes in chickens. Anim Sci J 2016 Jan 2
43 26045367 Reveal genes functionally associated with ACADS by a network study. Gene 2015 Sep 15 4
44 26989860 Effects of short-chain acyl-CoA dehydrogenase on cardiomyocyte apoptosis. J Cell Mol Med 2016 Jul 9
45 27051597 Clinical relevance of short-chain acyl-CoA dehydrogenase (SCAD) deficiency: Exploring the role of new variants including the first SCAD-disease-causing allele carrying a synonymous mutation. BBA Clin 2016 Jun 2
46 28018444 Compound heterozygous mutations of <i>ACADS</i> gene in newborn with short chain acyl-CoA dehydrogenase deficiency: case report and literatures review. Korean J Pediatr 2016 Nov 1
47 28095627 Melatonin regulates lipid metabolism in porcine oocytes. J Pineal Res 2017 Mar 1
48 28507299 The Fatty Acid β-Oxidation Pathway is Activated by Leucine Deprivation in HepG2 Cells: A Comparative Proteomics Study. Sci Rep 2017 May 15 2
49 30316772 Functional annotation of putative fadE9 of Mycobacterium tuberculosis as isobutyryl-CoA dehydrogenase involved in valine catabolism. Int J Biol Macromol 2019 Feb 1 7
50 31120904 Dynamic modelling of an ACADS genotype in fatty acid oxidation - Application of cellular models for the analysis of common genetic variants. PLoS One 2019 13