Anti-GATM Antibody Picoband®

SKU: A02754-1

Size: 100 μg

Storage: At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,IHC,IF,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
Immunohistochemistry (Paraffin-embedded Section), 2-5 μg/ml, Human, Rat
Immunofluorescence, 5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: GATM

Specificity:

Background: Glycine amidinotransferase, mitochondrial is an enzyme that in humans is encoded by the GATM gene. 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.

Immunogen: E.coli-derived human GATM recombinant protein (Position: M1-E279). Human GATM shares 95.3% and 95% amino acid (aa) sequence identity with mouse and rat GATM, respectively.

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Battini, R., Leuzzi, V., Carducci, C., Tosetti, M., Bianchi, M. C., Item, C. B., Stockler-Ipsiroglu, S., Cioni, G. Creatine depletion in a new case with AGAT deficiency: clinical and genetic study in a large pedigree. Molec. Genet. Metab. 77: 326-331, 2002. 2. Bianchi, M. C., Tosetti, M., Fornai, F., Alessandri, M. G., Cipriani, P., De Vito, G., Canapicchi, R. Reversible brain creatine deficiency in two sisters with normal blood creatine level. Ann. Neurol. 47: 511-513, 2000. 3. Choe, C., Nabuurs, C., Stockebrand, M. C., Neu, A., Nunes, P., Morellini, F., Sauter, K., Schillemeit, S., Hermans-Borgmeyer, I., Marescau, B., Heerschap, A., Isbrandt, D. L-arginine:glycine amidinotransferase deficiency protects from metabolic syndrome. Hum. Molec. Genet. 22: 110-123, 2013. Note: Erratum: Hum. Molec. Genet.: 22: 4030 only, 2013.

Uniprot ID: P50440

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 48 kDa

Calculated Molecular Weight: 48 kDa

Gene ID: 2628

Protein Name: Glycine amidinotransferase, mitochondrial

Gene Full Name: glycine amidinotransferase

Synonyms: AGAT; AT; GATM; Transamidinase