NP_000150.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
47,355 Da
NCBI Official Full Name
glutaryl-CoA dehydrogenase, mitochondrial isoform a
NCBI Official Synonym Full Names
glutaryl-CoA dehydrogenase
NCBI Official Synonym Symbols
NCBI Protein Information
glutaryl-CoA dehydrogenase, mitochondrial
UniProt Protein Name
Glutaryl-CoA dehydrogenase, mitochondrial
UniProt Synonym Gene Names
UniProt Entry Name
GCDH_HUMAN
NCBI Summary for GCDH
The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family. It catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I. Alternative splicing of this gene results in multiple transcript variants. A related pseudogene has been identified on chromosome 12. [provided by RefSeq, Mar 2013]
UniProt Comments for GCDH
GCDH: Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive. Defects in GCDH are the cause of glutaric aciduria type 1 (GA1). GA1 is an autosomal recessive metabolic disorder characterized by progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia. Belongs to the acyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 1.3.8.6; Mitochondrial; Amino Acid Metabolism - tryptophan; Amino Acid Metabolism - lysine degradation; Lipid Metabolism - fatty acid; Oxidoreductase
Chromosomal Location of Human Ortholog: 19p13.2
Cellular Component: mitochondrial inner membrane; mitochondrial matrix; mitochondrion
Molecular Function: acyl-CoA binding; electron carrier activity; FAD binding; glutaryl-CoA dehydrogenase activity
Biological Process: acyl-CoA biosynthetic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; lipid homeostasis; lysine catabolic process; tryptophan metabolic process
Disease: Glutaric Acidemia I
Research Articles on GCDH
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Products associated with anti-GCDH antibody
Pathways associated with anti-GCDH antibody
Products by Pathway |
Pathway Diagram |
Fatty Acid Beta Oxidation Pathway antibodies |
Fatty Acid Beta Oxidation Pathway Diagram |
Fatty Acid Degradation Pathway antibodies |
Fatty Acid Degradation Pathway Diagram |
Fatty Acid Degradation Pathway antibodies |
Fatty Acid Degradation Pathway Diagram |
Histidine, Lysine, Phenylalanine, Tyrosine, Proline And Tryptophan Catabolism Pathway antibodies |
Histidine, Lysine, Phenylalanine, Tyrosine, Proline And Tryptophan Catabolism Pathway Diagram |
Lysine Catabolism Pathway antibodies |
Lysine Catabolism Pathway Diagram |
Lysine Degradation Pathway antibodies |
Lysine Degradation Pathway Diagram |
Lysine Degradation Pathway antibodies |
Lysine Degradation Pathway Diagram |
Lysine Degradation, Lysine => Saccharopine => Acetoacetyl-CoA Pathway antibodies |
Lysine Degradation, Lysine => Saccharopine => Acetoacetyl-CoA Pathway Diagram |
Lysine Degradation, Lysine => Saccharopine => Acetoacetyl-CoA Pathway antibodies |
Lysine Degradation, Lysine => Saccharopine => Acetoacetyl-CoA Pathway Diagram |
Metabolic Pathways antibodies |
Metabolic Pathways Diagram |
Diseases associated with anti-GCDH antibody
Organs/Tissues associated with anti-GCDH antibody
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