NP_001305829.1
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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
46,575 Da
NCBI Official Full Name
glutamate dehydrogenase 1, mitochondrial isoform b
NCBI Official Synonym Full Names
glutamate dehydrogenase 1
NCBI Protein Information
glutamate dehydrogenase 1, mitochondrial
UniProt Protein Name
Glutamate dehydrogenase 1, mitochondrial
UniProt Synonym Gene Names
NCBI Summary for GDH
This gene encodes glutamate dehydrogenase, which is a mitochondrial matrix enzyme that catalyzes the oxidative deamination of glutamate to alpha-ketoglutarate and ammonia. This enzyme has an important role in regulating amino acid-induced insulin secretion. It is allosterically activated by ADP and inhibited by GTP and ATP. Activating mutations in this gene are a common cause of congenital hyperinsulinism. Alternative splicing of this gene results in multiple transcript variants. The related glutamate dehydrogenase 2 gene on the human X-chromosome originated from this gene via retrotransposition and encodes a soluble form of glutamate dehydrogenase. Related pseudogenes have been identified on chromosomes 10, 18 and X. [provided by RefSeq, Jan 2016]
UniProt Comments for GDH
GLUD1: May be involved in learning and memory reactions by increasing the turnover of the excitatory neurotransmitter glutamate. Homohexamer. Subject to allosteric regulation. Activated by ADP. Inhibited by GTP and ATP. ADP can occupy the NADH binding site and activate the enzyme. Belongs to the Glu/Leu/Phe/Val dehydrogenases family.
Protein type: Amino Acid Metabolism - alanine, aspartate and glutamate; Amino Acid Metabolism - arginine and proline; EC 1.4.1.3; Energy Metabolism - nitrogen; Mitochondrial; Other Amino Acids Metabolism - D-Glutamine and D-glutamate; Oxidoreductase
Chromosomal Location of Human Ortholog: 10q23.2
Cellular Component: cytoplasm; mitochondrial matrix; mitochondrion
Molecular Function: ADP binding; ATP binding; glutamate dehydrogenase [NAD(P)+] activity; glutamate dehydrogenase activity; GTP binding; identical protein binding; NAD+ binding; protein binding
Biological Process: amino acid biosynthetic process; glutamate biosynthetic process; glutamate catabolic process; glutamine metabolic process; mitochondrion organization; positive regulation of insulin secretion; substantia nigra development; tricarboxylic acid metabolic process
Disease: Hyperinsulinemic Hypoglycemia, Familial, 6
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Products associated with anti-GDH antibody
Pathways associated with anti-GDH antibody
Diseases associated with anti-GDH antibody
Organs/Tissues associated with anti-GDH antibody
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