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AGL 抗体 (AA 357-387)

Cited in 10+ publications. This 兔 多克隆 anti-AGL antibody (Clone RB4976) specifically detects AGL in WB 和 IF. The antibody is reactive with 人 samples.
产品编号 ABIN1882062
发货至: 中国
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Quick Overview for AGL 抗体 (AA 357-387) (ABIN1882062)

抗原

See all AGL 抗体
AGL (Amylo-alpha-1, 6-Glucosidase, 4-alpha-Glucanotransferase (AGL))

适用

  • 46
  • 14
  • 12

宿主

  • 46
  • 2

克隆类型

  • 35
  • 13
多克隆

标记

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  • 2
  • 2
  • 2
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  • 1
  • 1
This AGL antibody is un-conjugated

应用范围

  • 31
  • 31
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Western Blotting (WB), Immunofluorescence (IF)

克隆位点

RB4976
  • 抗原表位

    • 8
    • 7
    • 4
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    AA 357-387

    纯化方法

    This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.

    免疫原

    This AGL antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 357-387 amino acids from the Central region of human AGL.

    亚型

    Ig Fraction
  • 应用备注

    IF: 1:10~50. WB: 1:1000

    限制

    仅限研究用
  • 状态

    Liquid

    缓冲液

    Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.

    储存液

    Sodium azide

    注意事项

    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

    储存条件

    4 °C,-20 °C

    有效期

    6 months
  • Irimia, Tagliabracci, Meyer, Segvich, DePaoli-Roach, Roach: "Muscle glycogen remodeling and glycogen phosphate metabolism following exhaustive exercise of wild type and laforin knockout mice." in: The Journal of biological chemistry, (2015) (PubMed).

    Guin, Ru, Agarwal, Ritterson Lew, Owens, Comi, Theodorescu: "Loss of glycogen debranching enzyme AGL drives bladder tumor growth via induction of hyaluronic acid synthesis." in: Clinical cancer research : an official journal of the American Association for Cancer Research, (2015) (PubMed).

    DePaoli-Roach, Tagliabracci, Segvich, Meyer, Irimia, Roach: "Genetic depletion of the malin E3 ubiquitin ligase in mice leads to lafora bodies and the accumulation of insoluble laforin." in: The Journal of biological chemistry, Vol. 285, Issue 33, pp. 25372-81, (2010) (PubMed).

    Parker, Kong, Walsh, Salajegheh, Moghadaszadeh, Amato, Nazareno, Lin, Krastins, Sarracino, Beggs, Pinkus, Greenberg: "Fast-twitch sarcomeric and glycolytic enzyme protein loss in inclusion body myositis." in: Muscle & nerve, Vol. 39, Issue 6, pp. 739-53, (2009) (PubMed).

    Tagliabracci, Girard, Segvich, Meyer, Turnbull, Zhao, Minassian, Depaoli-Roach, Roach: "Abnormal metabolism of glycogen phosphate as a cause for Lafora disease." in: The Journal of biological chemistry, Vol. 283, Issue 49, pp. 33816-25, (2008) (PubMed).

    Cheng, Zhang, Gentry, Worby, Dixon, Saltiel: "A role for AGL ubiquitination in the glycogen storage disorders of Lafora and Cori's disease." in: Genes & development, Vol. 21, Issue 19, pp. 2399-409, (2007) (PubMed).

    Horinishi, Okubo, Tang, Hui, To, Mabuchi, Okada, Mabuchi, Murase: "Mutational and haplotype analysis of AGL in patients with glycogen storage disease type III." in: Journal of human genetics, Vol. 47, Issue 2, pp. 55-9, (2002) (PubMed).

    Bao, Dawson, Chen: "Human glycogen debranching enzyme gene (AGL): complete structural organization and characterization of the 5' flanking region." in: Genomics, Vol. 38, Issue 2, pp. 155-65, (1997) (PubMed).

    Hansen, Lundin, Markussen, Thorsby: "T cell receptor usage by HLA-DQw8-specific T cell clones." in: International immunology, Vol. 4, Issue 8, pp. 931-4, (1992) (PubMed).

    Yang, Ding, Enghild, Bao, Chen: "Molecular cloning and nucleotide sequence of cDNA encoding human muscle glycogen debranching enzyme." in: The Journal of biological chemistry, Vol. 267, Issue 13, pp. 9294-9, (1992) (PubMed).

  • 抗原

    AGL (Amylo-alpha-1, 6-Glucosidase, 4-alpha-Glucanotransferase (AGL))

    别名

    AGL

    背景

    AGL is a glycogen debrancher enzyme which is involved in glycogen degradation. This enzyme has two independent catalytic activities which occur at different sites on the protein: a 4-alpha-glucotransferase activity and a amylo-1,6-glucosidase activity. Mutations in the AGL gene are associated with glycogen storage disease although a wide range of enzymatic and clinical variability occurs which may be due to tissue-specific alternative splicing.

    分子量

    174764

    NCBI登录号

    NP_000019, NP_000633, NP_000634, NP_000635, NP_000636, NP_000637

    UniProt

    P35573

    途径

    Cellular Glucan Metabolic Process
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