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GCSH 抗体 (AA 101-173)

GCSH 适用: 人 WB, ELISA, IF (cc), IF (p), IHC (fro), IHC (p), ICC 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN1713812
发货至: 中国
  • 抗原 See all GCSH 抗体
    GCSH (Glycine Cleavage System H Protein (GCSH))
    抗原表位
    • 14
    • 8
    • 7
    • 6
    • 3
    • 2
    AA 101-173
    适用
    • 43
    • 11
    • 11
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    宿主
    • 41
    • 2
    克隆类型
    • 42
    • 1
    多克隆
    标记
    • 12
    • 4
    • 3
    • 3
    • 2
    • 2
    • 2
    • 2
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    This GCSH antibody is un-conjugated
    应用范围
    • 35
    • 18
    • 12
    • 12
    • 3
    • 2
    • 2
    • 2
    • 1
    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)
    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
    Top Product
    Discover our top product GCSH Primary Antibody
  • 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 (GCSH 产品)
    别名
    GCE antibody, NKH antibody, GCSH antibody, H-PROTEIN antibody, 1100001L02Rik antibody, 5730591C18Rik antibody, wu:fb14d10 antibody, wu:fw84b08 antibody, zgc:112535 antibody, BA5228 antibody, gce antibody, gcsh antibody, nkh antibody, wu:fd56h06 antibody, zgc:110557 antibody, zgc:92732 antibody, glycine cleavage system protein H antibody, glycine cleavage system protein H (aminomethyl carrier) antibody, glycine cleavage system lipoyl carrier protein GcvH antibody, glycine cleavage system H protein antibody, glycine cleavage system protein H (aminomethyl carrier), a antibody, glycine cleavage system carrier of aminomethyl moiety GcvH antibody, leucine rich repeat containing 34 antibody, glycine cleavage system protein H (aminomethyl carrier) L homeolog antibody, glycine cleavage system protein H (aminomethyl carrier), b antibody, GCSH antibody, Gcsh antibody, gcvH-2 antibody, gcvH antibody, gcsha antibody, LRRC34 antibody, gcsh.L antibody, gcshb antibody
    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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