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GCSH 抗体 (AA 101-173) (FITC)

This anti-GCSH antibody is a 兔 多克隆 antibody detecting GCSH in WB, IF (cc) 和 IF (p). Suitable for 人.
产品编号 ABIN1709514
发货至: 中国

Quick Overview for GCSH 抗体 (AA 101-173) (FITC) (ABIN1709514)

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 conjugated to FITC

应用范围

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Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
  • 抗原表位

    • 14
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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

    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200

    限制

    仅限研究用
  • 状态

    Liquid

    浓度

    1 μg/μL

    缓冲液

    Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % 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

    -20 °C

    储存方法

    Store at -20°C. Aliquot into multiple vials to 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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