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1.3.8.5: short-chain 2-methylacyl-CoA dehydrogenase

This is an abbreviated version!
For detailed information about short-chain 2-methylacyl-CoA dehydrogenase, go to the full flat file.

Word Map on EC 1.3.8.5

Reaction

2-methylbutanoyl-CoA
+
electron-transfer flavoprotein
=
(E)-2-methylbut-2-enoyl-CoA
 +
reduced electron-transfer flavoprotein
 +
H+

Synonyms

2-methyl branched chain acyl-CoA dehydrogenase, 2-Methyl branched-chain acyl-CoA dehydrogenase, 2-Methyl branched-chain enoyl CoA reductase, 2-Methyl branched-chain enoyl-CoA reductase, 2-methylbutyryl-CoA dehydrogenase, 2MBCD, branched chain acyl-CoA dehydrogenase, EC 1.3.99.12, ECR, Methyl branched-chain enoyl-CoA reductase, Reductase, methyl branched-chain enoyl coenzyme A, SBCAD, SBCADD, short/branched chain acyl-CoA dehydrogenase

ECTree

     1 Oxidoreductases
         1.3 Acting on the CH-CH group of donors
             1.3.8 With a flavin as acceptor
                1.3.8.5 short-chain 2-methylacyl-CoA dehydrogenase

Disease

topprint

Disease on EC 1.3.8.5 - short-chain 2-methylacyl-CoA dehydrogenase

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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
acyl-coa dehydrogenase deficiency
2-methylbutyryl-CoA dehydrogenase deficiency associated with autism and mental retardation: a case report.
Acylglycine Analysis by Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry (UPLC-MS/MS).
Biochemical, Clinical, and Genetic Characteristics of Short/Branched Chain Acyl-CoA Dehydrogenase Deficiency in Chinese Patients by Newborn Screening.
Prevalence and mutation analysis of short/branched chain acyl-CoA dehydrogenase deficiency (SBCADD) detected on newborn screening in Wisconsin.
Intellectual Disability
2-methylbutyryl-CoA dehydrogenase deficiency associated with autism and mental retardation: a case report.
Maple Syrup Urine Disease
Advances and challenges in the treatment of branched-chain amino/keto acid metabolic defects.
medium-chain acyl-coa dehydrogenase deficiency
Acylglycine Analysis by Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry (UPLC-MS/MS).
methylcrotonoyl-coa carboxylase deficiency
Acylglycine Analysis by Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry (UPLC-MS/MS).
Multiple Acyl Coenzyme A Dehydrogenase Deficiency
Acylglycine Analysis by Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry (UPLC-MS/MS).
Propionic Acidemia
Acylglycine Analysis by Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry (UPLC-MS/MS).
Advances and challenges in the treatment of branched-chain amino/keto acid metabolic defects.
Seizures
Short/branched-chain acyl-CoA dehydrogenase deficiency due to an IVS3+3A>G mutation that causes exon skipping.
short-chain 2-methylacyl-coa dehydrogenase deficiency
2-methylbutyryl-CoA dehydrogenase deficiency associated with autism and mental retardation: a case report.
2-methylbutyryl-CoA dehydrogenase deficiency in Hmong infants identified by expanded newborn screen.
2-Methylbutyrylglycine induces lipid oxidative damage and decreases the antioxidant defenses in rat brain.
Acylglycine Analysis by Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry (UPLC-MS/MS).
Advances and challenges in the treatment of branched-chain amino/keto acid metabolic defects.
Biochemical, Clinical, and Genetic Characteristics of Short/Branched Chain Acyl-CoA Dehydrogenase Deficiency in Chinese Patients by Newborn Screening.
Characterization of new ACADSB gene sequence mutations and clinical implications in patients with 2-methylbutyrylglycinuria identified by newborn screening.
Clinical, biochemical, and molecular spectrum of short/branched-chain acyl-CoA dehydrogenase deficiency: two new cases and review of literature.
Inborn errors of isoleucine degradation: a review.
Prevalence and mutation analysis of short/branched chain acyl-CoA dehydrogenase deficiency (SBCADD) detected on newborn screening in Wisconsin.
Prospective diagnosis of 2-methylbutyryl-CoA dehydrogenase deficiency in the Hmong population by newborn screening using tandem mass spectrometry.
UPLC-MS/MS analysis of C5-acylcarnitines in dried blood spots.
short-chain acyl-coa dehydrogenase deficiency
Acylglycine Analysis by Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry (UPLC-MS/MS).