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GATM Blocking Peptide(C-term)
Synthetic peptide
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
| Primary Accession | P50440 |
|---|---|
| Other Accession | P50442, P50441, Q9D964, Q4R806, Q2HJ74, NP_001473.1 |
| Gene ID | 2628 |
|---|---|
| Other Names | Glycine amidinotransferase, mitochondrial, L-arginine:glycine amidinotransferase, Transamidinase, GATM, AGAT |
| Target/Specificity | The synthetic peptide sequence is selected from aa 365-378 of HUMAN GATM |
| Format | Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed. |
| Storage | Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C. |
| Precautions | This product is for research use only. Not for use in diagnostic or therapeutic procedures. |
| Name | GATM |
|---|---|
| Synonyms | AGAT |
| Function | Transamidinase that catalyzes the transfer of the amidino group of L-arginine onto the amino moiety of acceptor metabolites such as glycine, beta-alanine, gamma-aminobutyric acid (GABA) and taurine yielding the corresponding guanidine derivatives (PubMed:3800397, PubMed:16820567, PubMed:36543883, PubMed:27233232). Catalyzes the rate- limiting step of creatine biosynthesis, namely the transfer of the amidino group from L-arginine to glycine to generate guanidinoacetate, which is then methylated by GAMT to form creatine. Provides creatine as a source for ATP generation in tissues with high energy demands, in particular skeletal muscle, heart and brain (PubMed:3800397, PubMed:36543883, PubMed:9266688, PubMed:27233232) (Probable). |
| Cellular Location | [Isoform 1]: Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side. Note=Probably attached to the outer side of the inner membrane |
| Tissue Location | Expressed in brain, heart, kidney, liver, lung, salivary gland and skeletal muscle tissue, with the highest expression in kidney. Biallelically expressed in placenta and fetal tissues |
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Provided below are standard protocols that you may find useful for product applications.
Background
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 mental retardation, language impairment, and behavioral disorders.
References
Kottgen, A., et al. Nat. Genet. 42(5):376-384(2010)
Kottgen, A., et al. Nat. Genet. 41(6):712-717(2009)
Lion-Francois, L., et al. Neurology 67(9):1713-1714(2006)
Cullen, M.E., et al. Circulation 114 (1 SUPPL), I16-I20 (2006) :
Battini, R., et al. J. Pediatr. 148(6):828-830(2006)
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