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GCSH antibody (AA 101-173)

GCSH Reactivity: Human WB, ELISA, IF (cc), IF (p), IHC (fro), IHC (p), ICC Host: Rabbit Polyclonal unconjugated
Catalog No. ABIN1713812
  • Target See all GCSH Antibodies
    GCSH (Glycine Cleavage System H Protein (GCSH))
    Binding Specificity
    • 14
    • 8
    • 7
    • 6
    • 3
    • 2
    AA 101-173
    Reactivity
    • 43
    • 11
    • 11
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    Human
    Host
    • 41
    • 2
    Rabbit
    Clonality
    • 42
    • 1
    Polyclonal
    Conjugate
    • 12
    • 4
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This GCSH antibody is un-conjugated
    Application
    • 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)
    Predicted Reactivity
    Human,Mouse,Rat,Horse
    Purification
    Purified by Protein A.
    Immunogen
    KLH conjugated synthetic peptide derived from human GCSH
    Isotype
    IgG
    Top Product
    Discover our top product GCSH Primary Antibody
  • Application Notes
    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
    Restrictions
    For Research Use only
  • Format
    Liquid
    Concentration
    1 μg/μL
    Buffer
    0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.
    Preservative
    ProClin
    Precaution of Use
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    Storage
    4 °C,-20 °C
    Storage Comment
    Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
    Expiry Date
    12 months
  • Target
    GCSH (Glycine Cleavage System H Protein (GCSH))
    Alternative Name
    GCSH (GCSH Products)
    Synonyms
    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
    Background

    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.

    Gene ID
    2653
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