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Glycogenin 1 抗体 (AA 1-73)

Cited in 6+ publications. This 小鼠 单克隆 anti-Glycogenin 1 antibody (Clone 3B5) specifically detects Glycogenin 1 in WB, ELISA 和 IHC (p). The antibody is reactive with 人 samples.
产品编号 ABIN561206
发货至: 中国
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Quick Overview for Glycogenin 1 抗体 (AA 1-73) (ABIN561206)

抗原

See all Glycogenin 1 (GYG1) 抗体
Glycogenin 1 (GYG1)

适用

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宿主

  • 24
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小鼠

克隆类型

  • 26
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单克隆

标记

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This Glycogenin 1 antibody is un-conjugated

应用范围

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

克隆位点

3B5
  • 抗原表位

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    AA 1-73

    原理

    Mouse monoclonal antibody raised against a partial recombinant GYG1.

    序列

    MTDQAFVTLT TNDAYAKGAL VLGSSLKQHR TTRRLVVLAT PQVSDSMRKV LETVFDEVIM VDVLDSGDSA HLT

    交叉反应

    人, 大鼠

    产品特性

    Antibody Reactive Against Recombinant Protein.

    免疫原

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

    亚型

    IgG2a
  • 应用备注

    Optimal working dilution should be determined by the investigator.

    限制

    仅限研究用
  • 缓冲液

    In 1x PBS, pH 7.4

    注意事项

    Aliquot to avoid repeated freezing and thawing.

    储存条件

    -20 °C

    储存方法

    Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
  • 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).

  • 抗原

    Glycogenin 1 (GYG1)

    别名

    GYG1

    背景

    Full Gene Name: glycogenin 1
    Synonyms: GYG

    基因ID

    2992

    NCBI登录号

    NM_004130

    途径

    Cellular Glucan Metabolic Process
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