NP_001003941.1
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NCBI GenBank Nucleotide #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
48,180 Da
NCBI Official Full Name
2-oxoglutarate dehydrogenase, mitochondrial isoform 2
NCBI Official Synonym Full Names
oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)
NCBI Protein Information
2-oxoglutarate dehydrogenase, mitochondrial; OGDC-E1; oxoglutarate decarboxylase; 2-oxoglutarate dehydrogenase complex component E1; oxoglutarate dehydrogenase (succinyl-transferring)
UniProt Protein Name
2-oxoglutarate dehydrogenase, mitochondrial
UniProt Synonym Protein Names
2-oxoglutarate dehydrogenase complex component E1; OGDC-E1; Alpha-ketoglutarate dehydrogenase
UniProt Synonym Gene Names
UniProt Entry Name
ODO1_HUMAN
NCBI Summary for OGDH
This gene encodes one subunit of the 2-oxoglutarate dehydrogenase complex. This complex catalyzes the overall conversion of 2-oxoglutarate (alpha-ketoglutarate) to succinyl-CoA and CO(2) during the Krebs cycle. The protein is located in the mitochondrial matrix and uses thiamine pyrophosphate as a cofactor. A congenital deficiency in 2-oxoglutarate dehydrogenase activity is believed to lead to hypotonia, metabolic acidosis, and hyperlactatemia. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Sep 2009]
UniProt Comments for OGDH
OGDH: The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2- oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3). Belongs to the alpha-ketoglutarate dehydrogenase family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: Amino Acid Metabolism - tryptophan; Oxidoreductase; Mitochondrial; Amino Acid Metabolism - lysine degradation; Carbohydrate Metabolism - citrate (TCA) cycle; EC 1.2.4.2
Chromosomal Location of Human Ortholog: 7p14-p13
Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial membrane; oxoglutarate dehydrogenase complex; cytosol
Molecular Function: metal ion binding; chaperone binding; heat shock protein binding; thiamin pyrophosphate binding; oxoglutarate dehydrogenase (succinyl-transferring) activity
Biological Process: generation of precursor metabolites and energy; glycolysis; striatum development; tricarboxylic acid cycle; succinyl-CoA metabolic process; hippocampus development; pyramidal neuron development; thalamus development; 2-oxoglutarate metabolic process; cerebellar cortex development; cellular metabolic process; tangential migration from the subventricular zone to the olfactory bulb; NADH metabolic process; lysine catabolic process
Disease: Alpha-ketoglutarate Dehydrogenase Deficiency
Research Articles on OGDH
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Products associated with OGDH elisa kit
Pathways associated with OGDH elisa kit
Products by Pathway |
Pathway Diagram |
2-oxoglutarate Decarboxylation To Succinyl-CoA Pathway antibodies |
2-oxoglutarate Decarboxylation To Succinyl-CoA Pathway Diagram |
2-oxoglutarate Decarboxylation To Succinyl-CoA Pathway antibodies |
2-oxoglutarate Decarboxylation To Succinyl-CoA Pathway Diagram |
Carbon Metabolism Pathway antibodies |
Carbon Metabolism Pathway Diagram |
Carbon Metabolism Pathway antibodies |
Carbon Metabolism Pathway Diagram |
Citrate Cycle (TCA Cycle) Pathway antibodies |
Citrate Cycle (TCA Cycle) Pathway Diagram |
Citrate Cycle (TCA Cycle) Pathway antibodies |
Citrate Cycle (TCA Cycle) Pathway Diagram |
Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway antibodies |
Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway Diagram |
Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway antibodies |
Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway Diagram |
Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway antibodies |
Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway Diagram |
Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway antibodies |
Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway Diagram |
Diseases associated with OGDH elisa kit
Organs/Tissues associated with OGDH elisa kit
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