{"product_id":"anti-gatm-antibody-picoband-r","title":"Anti-GATM Antibody Picoband®","description":"\u003cp\u003e\u003cb\u003eSize\u003c\/b\u003e: 100 μg\u003c\/p\u003e\u003cp\u003e\u003cb\u003eStorage\u003c\/b\u003e: At -20°C for one year from date of receipt. \nAfter reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months.\nAvoid repeated freezing and thawing.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eForm\u003c\/b\u003e: Lyophilized\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReactivity\u003c\/b\u003e: Human,Mouse,Rat\u003c\/p\u003e\u003cp\u003e\u003cb\u003eApplications\u003c\/b\u003e: WB,IHC,IF,Flow Cytometry,ELISA\u003c\/p\u003e\u003cp\u003e\u003cb\u003eApplication Details\u003c\/b\u003e: Western blot, 0.25-0.5 μg\/ml, Human, Mouse, Rat\u003cbr\u003e\nImmunohistochemistry (Paraffin-embedded Section), 2-5 μg\/ml, Human, Rat\u003cbr\u003e\nImmunofluorescence, 5 μg\/ml, Human\u003cbr\u003e\nFlow Cytometry (Fixed), 1-3 μg\/1x10\u003csup\u003e6\u003c\/sup\u003e cells, Human\u003cbr\u003e\nELISA, 0.1-0.5 μg\/ml, -\u003cbr\u003e\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene Name\u003c\/b\u003e: GATM\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSpecificity\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eBackground\u003c\/b\u003e: 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.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eImmunogen\u003c\/b\u003e: 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.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eClonality\u003c\/b\u003e: Polyclonal\u003c\/p\u003e\u003cp\u003e\u003cb\u003eContents\u003c\/b\u003e: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.\u003c\/p\u003e\u003cp\u003e\u003cb\u003ePurification\u003c\/b\u003e: Immunogen affinity purified.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReconstitution\u003c\/b\u003e: Adding 0.2 ml of distilled water will yield a concentration of 500 μg\/ml.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReference\u003c\/b\u003e: 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.\n2. 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.\n3. 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.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eUniprot ID\u003c\/b\u003e: P50440\u003c\/p\u003e\u003cp\u003e\u003cb\u003eHost\u003c\/b\u003e: Rabbit\u003c\/p\u003e\u003cp\u003e\u003cb\u003eConcentration\u003c\/b\u003e: Adding 0.2 ml of distilled water will yield a concentration of 500 μg\/ml.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eConjugate\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eCross Reactivity\u003c\/b\u003e: No cross-reactivity with other proteins\u003c\/p\u003e\u003cp\u003e\u003cb\u003eIsotype\u003c\/b\u003e: Rabbit IgG\u003c\/p\u003e\u003cp\u003e\u003cb\u003ePhospho_site\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eClone Number\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eObserved Molecular Weight\u003c\/b\u003e: 48 kDa\u003c\/p\u003e\u003cp\u003e\u003cb\u003eCalculated Molecular Weight\u003c\/b\u003e: 48 kDa\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene ID\u003c\/b\u003e: 2628\u003c\/p\u003e\u003cp\u003e\u003cb\u003eProtein Name\u003c\/b\u003e: Glycine amidinotransferase, mitochondrial\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene Full Name\u003c\/b\u003e: glycine amidinotransferase\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSynonyms\u003c\/b\u003e: AGAT; AT; GATM; Transamidinase\u003c\/p\u003e","brand":"GeneBio Systems","offers":[{"title":"Default Title","offer_id":48788015841380,"sku":"A02754-1","price":618.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0558\/8588\/9636\/files\/no_image_default_image-jpeg_969b2b00-7d22-475b-b312-ed8192f7b287.jpg?v=1784406723","url":"https:\/\/www.genebiosystems.com\/en-us\/products\/anti-gatm-antibody-picoband-r","provider":"GeneBio ","version":"1.0","type":"link"}