Glycogenin 1 Antikörper (AA 1-73)

Produktnummer ABIN561206

Produktdetails anti-Glycogenin 1 Antikörper

Target: Glycogenin 1 (GYG1)

Bindungsspezifität: AA 1-73

Reaktivität: Human

Wirt: Maus

Klonalität: Monoklonal

Konjugat: Dieser Glycogenin 1 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Verwendungszweck: Mouse monoclonal antibody raised against a partial recombinant GYG1.

Sequenz: MTDQAFVTLT TNDAYAKGAL VLGSSLKQHR TTRRLVVLAT PQVSDSMRKV LETVFDEVIM VDVLDSGDSA HLT

Kreuzreaktivität: Human, Ratte

Produktmerkmale: Antibody Reactive Against Recombinant Protein.

Immunogen: GYG1 (NP_004121, 1 a.a. ~ 73 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Klon: 3B5

Isotyp: IgG2a

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Buffer: In 1x PBS, pH 7.4

Handhabung: Aliquot to avoid repeated freezing and thawing.

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.

Referenzen für anti-Glycogenin 1 Antikörper (ABIN561206)

Tasca, Fattori, Monforte, Hedberg-Oldfors, Sabatelli, Udd, Boldrini, Bertini, Ricci, Oldfors: "Start codon mutation of GYG1 causing late-onset polyglucosan body myopathy with nemaline rods." in: Journal of neurology, Vol. 263, Issue 10, pp. 2133-5, (2016) (PubMed).

Hedberg-Oldfors, Glamuzina, Ruygrok, Anderson, Elliott, Watkinson, Occleshaw, Abernathy, Turner, Kingston, Murphy, Oldfors: "Cardiomyopathy as presenting sign of glycogenin-1 deficiency-report of three cases and review of the literature." in: Journal of inherited metabolic disease, Vol. 40, Issue 1, pp. 139-149, (2016) (PubMed).

Nilsson, Halim, Larsson, Moslemi, Oldfors, Larson, Nilsson: "LC-MS/MS characterization of combined glycogenin-1 and glycogenin-2 enzymatic activities reveals their self-glucosylation preferences." in: Biochimica et biophysica acta, Vol. 1844, Issue 2, pp. 398-405, (2014) (PubMed).

Malfatti, Nilsson, Hedberg-Oldfors, Hernandez-Lain, Michel, Dominguez-Gonzalez, Viennet, Akman, Kornblum, Van den Bergh, Romero, Engel, DiMauro, Oldfors: "A new muscle glycogen storage disease associated with glycogenin-1 deficiency." in: Annals of neurology, Vol. 76, Issue 6, pp. 891-8, (2014) (PubMed).

Taylor, Meyers, Phipps, Kishnani, Cheng, Scheule, Moreland: "Dysregulation of multiple facets of glycogen metabolism in a murine model of Pompe disease." in: PLoS ONE, Vol. 8, Issue 2, pp. e56181, (2013) (PubMed).

Nilsson, Halim, Moslemi, Pedersen, Nilsson, Larson, Oldfors: "Molecular pathogenesis of a new glycogenosis caused by a glycogenin-1 mutation." in: Biochimica et biophysica acta, Vol. 1822, Issue 4, pp. 493-9, (2012) (PubMed).

Details zu Glycogenin 1

Target: Glycogenin 1 (GYG1)

Andere Bezeichnung: GYG1 (GYG1 Produkte)

Hintergrund: Full Gene Name: glycogenin 1
Synonyms: GYG

Gen-ID: 2992

NCBI Accession: NM_004130

Pathways: Cellular Glucan Metabolic Process