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Rabbit Anti-GLUD1 Recombinant Antibody (CBLG1-2825) (CBMAB-G0537-LY)


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Summary

Host Animal
Rabbit
Specificity
Human
Clone
CBLG1-2825
Antibody Isotype
IgG
Application
ICC/IF

Basic Information

Specificity
Human
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Glutamate Dehydrogenase 1
Introduction
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]
Entrez Gene ID
2746
UniProt ID
P00367
Alternative Names
Glutamate Dehydrogenase 1; EC 1.4.1.3; GLUD; Epididymis Tissue Sperm Binding Protein Li 18mP; Glutamate Dehydrogenase 1; Mitochondrial; Glutamate Dehydrogenase (NAD(P)+);
Function
Mitochondrial glutamate dehydrogenase that catalyzes the conversion of L-glutamate into alpha-ketoglutarate. Plays a key role in glutamine anaplerosis by producing alpha-ketoglutarate, an important intermediate in the tricarboxylic acid cycle (PubMed:11032875, PubMed:16959573, PubMed:11254391, PubMed:16023112).

Plays a role in insulin homeostasis (PubMed:9571255, PubMed:11297618).

May be involved in learning and memory reactions by increasing the turnover of the excitatory neurotransmitter glutamate (By similarity).
Biological Process
Glutamate biosynthetic process Source: BHF-UCL
Glutamate catabolic process Source: UniProtKB
Glutamine metabolic process Source: UniProtKB
Positive regulation of insulin secretion Source: BHF-UCL
Substantia nigra development Source: UniProtKB
Tricarboxylic acid metabolic process Source: UniProtKB
Cellular Location
Endoplasmic reticulum; Mitochondrion. Mostly translocates into the mitochondria, only a small amount of the protein localizes to the endoplasmic reticulum.
Involvement in disease
Familial hyperinsulinemic hypoglycemia 6 (HHF6):
Familial hyperinsulinemic hypoglycemia [MIM:256450], also referred to as congenital hyperinsulinism, nesidioblastosis, or persistent hyperinsulinemic hypoglycemia of infancy (PPHI), is the most common cause of persistent hypoglycemia in infancy and is due to defective negative feedback regulation of insulin secretion by low glucose levels. In HHF6 elevated oxidation rate of glutamate to alpha-ketoglutarate stimulates insulin secretion in the pancreatic beta cells, while they impair detoxification of ammonium in the liver.
PTM
ADP-ribosylated by SIRT4, leading to inactivate glutamate dehydrogenase activity (PubMed:16959573). Stoichiometry shows that ADP-ribosylation occurs in one subunit per catalytically active homohexamer (PubMed:16023112).
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For research use only. Not intended for any clinical use.

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We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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