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Mouse Anti-GATM Recombinant Antibody (CBLG1-2624) (CBMAB-G2331-LY)

This product is antibody recognizes GATM. The antibody CBLG1-2624 immunoassay techniques such as: WB.
See all GATM antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBLG1-2624
Antibody Isotype
IgG1
Application
WB

Basic Information

Immunogen
Full length human recombinant protein of human GATM(NP_001473) produced in HEK293 cell
Specificity
Human
Antibody Isotype
IgG1
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
Buffer
50% glycerol
Preservative
0.02% NaN3
Concentration
0.41 mg/mL
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
Glycine Amidinotransferase
Introduction
This gene encodes a mitochondrial enzyme that belongs to the amidinotransferase family. This enzyme is involved in creatine biosynthesis, whereby it catalyzes the transfer of a guanido group from L-arginine to glycine, resulting in guanidinoacetic acid, the immediate precursor of creatine. Mutations in this gene cause arginine:glycine amidinotransferase deficiency, an inborn error of creatine synthesis characterized by cognitive disability, language impairment, and behavioral disorders. [provided by RefSeq, Jul 2008]
Entrez Gene ID
2628
UniProt ID
P50440
Alternative Names
Glycine Amidinotransferase; L-Arginine:Glycine Amidinotransferase; Transamidinase; EC 2.1.4.1; AGAT; Glycine Amidinotransferase (L-Arginine:Glycine Amidinotransferase);
Function
Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development. May be involved in the response to heart failure by elevating local creatine synthesis.
Biological Process
Creatine biosynthetic process Source: MGI
Creatine metabolic process Source: CAFA
Learning or memory Source: CAFA
Multicellular organism development Source: CAFA
Muscle atrophy Source: CAFA
Positive regulation of cold-induced thermogenesis Source: YuBioLab
Cellular Location
Isoform 1: Mitochondrion inner membrane. Probably attached to the outer side of the inner membrane.
Isoform 2: Cytoplasm
Involvement in disease
Cerebral creatine deficiency syndrome 3 (CCDS3):
An autosomal recessive disorder characterized by developmental delay/regression, mental retardation, severe disturbance of expressive and cognitive speech, and severe depletion of creatine/phosphocreatine in the brain. Most patients develop a myopathy characterized by muscle weakness and atrophy later in life.
Fanconi renotubular syndrome 1 (FRTS1):
A form of Fanconi renotubular syndrome, a disease due to a generalized dysfunction of the proximal kidney tubule resulting in decreased solute and water reabsorption. Patients have polydipsia and polyuria with phosphaturia, glycosuria and aminoaciduria. They may develop hypophosphatemic rickets or osteomalacia, acidosis and a tendency toward dehydration. Some eventually develop renal insufficiency. FRTS1 inheritance is autosomal dominant.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

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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