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GAA 抗体 (N-Term)

This anti-GAA antibody is a 兔 多克隆 antibody detecting GAA in WB 和 IHC. Suitable for 人, 小鼠, 大鼠, Cow, 犬, 豚鼠, 斑马鱼, 马 和 兔.
产品编号 ABIN2781771
发货至: 中国

Quick Overview for GAA 抗体 (N-Term) (ABIN2781771)

抗原

See all GAA 抗体
GAA (Glucosidase, Alpha, Acid (GAA))

适用

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人, 小鼠, 大鼠, Cow, 犬, 豚鼠, 斑马鱼, 马, 兔

宿主

  • 54
  • 6

克隆类型

  • 52
  • 8
多克隆

标记

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This GAA antibody is un-conjugated

应用范围

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Western Blotting (WB), Immunohistochemistry (IHC)
  • 抗原表位

    • 15
    • 7
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    N-Term

    序列

    FGVIVRRQLD GRVLLNTTVA PLFFADQFLQ LSTSLPSQYI TGLAEHLSPL

    预测反应

    Cow: 100%, Dog: 100%, Guinea Pig: 100%, Horse: 93%, Human: 100%, Mouse: 100%, Rabbit: 79%, Rat: 100%, Zebrafish: 83%

    产品特性

    This is a rabbit polyclonal antibody against GAA. It was validated on Western Blot using a cell lysate as a positive control.

    纯化方法

    Affinity Purified

    免疫原

    The immunogen is a synthetic peptide directed towards the N terminal region of human GAA
  • 应用备注

    Optimal working dilutions should be determined experimentally by the investigator.

    说明

    Antigen size: 952 AA

    限制

    仅限研究用
  • 状态

    Liquid

    浓度

    Lot specific

    缓冲液

    Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.

    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.

    注意事项

    Avoid repeated freeze-thaw cycles.

    储存条件

    -20 °C

    储存方法

    For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
  • 抗原

    GAA (Glucosidase, Alpha, Acid (GAA))

    别名

    GAA

    背景

    GAA is acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene.
    Alias Symbols: LYAG
    Protein Interaction Partner: UBC, SUMO1, NEDD8, NUMBL, STAT2, HIVEP1, EP300, SYNCRIP, MTHFD1, ILF3, HNRNPK, CDH2, CALU, FBXO6, NCF1,
    Protein Size: 952

    分子量

    98 kDa

    基因ID

    2548

    NCBI登录号

    NM_000152, NP_000143

    UniProt

    P10253

    途径

    Cellular Glucan Metabolic Process
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