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GCSH 抗体 (AA 101-173)

This 兔 多克隆 anti-GCSH antibody specifically detects GCSH in WB, ELISA, IF (cc), IF (p), IHC (fro), IHC (p) 和 ICC. The antibody is reactive with 人 samples.
产品编号 ABIN1713812
发货至: 中国
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Tel +86 (0512) 65829739 传真 +86 (010) 6788 5057

Quick Overview for GCSH 抗体 (AA 101-173) (ABIN1713812)

抗原

See all GCSH 抗体
GCSH (Glycine Cleavage System H Protein (GCSH))

适用

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宿主

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克隆类型

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多克隆

标记

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This GCSH antibody is un-conjugated

应用范围

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Western Blotting (WB), ELISA, Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Frozen Sections) (IHC (fro)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunocytochemistry (ICC)
  • 抗原表位

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    AA 101-173

    预测反应

    Human,Mouse,Rat,Horse

    纯化方法

    Purified by Protein A.

    免疫原

    KLH conjugated synthetic peptide derived from human GCSH

    亚型

    IgG
  • 应用备注

    WB 1:300-5000
    ELISA 1:500-1000
    IHC-P 1:200-400
    IHC-F 1:100-500
    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200
    ICC 1:100-500

    限制

    仅限研究用
  • 状态

    Liquid

    浓度

    1 μg/μL

    缓冲液

    0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.

    储存液

    ProClin

    注意事项

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

    储存条件

    4 °C,-20 °C

    储存方法

    Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

    有效期

    12 months
  • 抗原

    GCSH (Glycine Cleavage System H Protein (GCSH))

    别名

    GCSH

    背景

    Synonyms: GCE, GCSH, GCSH_HUMAN, Glycine cleavage system H protein, Glycine cleavage system H protein mitochondrial, Glycine cleavage system protein H aminomethyl carrier, Glycine cleavage system protein H, Lipoic acid containing protein, mitochondrial, Mitochondrial glycine cleavage system H protein, NKH.

    Background: GCSH is a 173 amino acid mitochondrial protein that contains one lipoyl-binding domain and belongs to the gcvH family. Defects in the gene encoding GCSH are the cause of glycine encephalopathy (GCE), an autosomal recessive disease that is also referred to as non-ketotic hyperglycinemia (NKH). Characterized by severe neurological symptoms, patients with GCE have a large amount of glycine accumulated in their body fluids. The gene encoding GCSH maps to human chromosome 16, which encodes over 900 genes and comprises nearly 3 % of the human genome.

    基因ID

    2653
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