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GAA antibody (N-Term)

GAA Reactivity: Human WB, IHC, IHC (p) Host: Rabbit Polyclonal RB18979 unconjugated
Catalog No. ABIN2845755
  • Target See all GAA Antibodies
    GAA (Glucosidase, Alpha, Acid (GAA))
    Binding Specificity
    • 15
    • 9
    • 6
    • 6
    • 4
    • 3
    • 3
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 174-203, N-Term
    Reactivity
    • 48
    • 22
    • 21
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    Human
    Host
    • 48
    • 4
    Rabbit
    Clonality
    • 48
    • 4
    Polyclonal
    Conjugate
    • 27
    • 5
    • 4
    • 3
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This GAA antibody is un-conjugated
    Application
    • 48
    • 19
    • 15
    • 13
    • 13
    • 8
    • 7
    • 5
    • 3
    • 2
    • 2
    • 1
    Western Blotting (WB), Immunohistochemistry (IHC), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))
    Purification
    This antibody is purified through a protein A column, followed by peptide affinity purification.
    Immunogen
    This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.
    Clone
    RB18979
    Isotype
    IgG
  • Application Notes
    WB: 1:2000. WB: 1:1000. WB: 1:1000. WB: 1:1000. IHC-P-Leica: 1:500. IHC-P-Leica: 1:500
    Restrictions
    For Research Use only
  • Format
    Liquid
    Buffer
    Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.
    Preservative
    Sodium azide
    Precaution of Use
    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
    Storage
    4 °C,-20 °C
    Storage Comment
    GAA Antibody (N-term) can be refrigerated at 2-8 °C for up to 6 months. For long term storage, place the at -20 °C.
    Expiry Date
    6 months
  • Target
    GAA (Glucosidase, Alpha, Acid (GAA))
    Alternative Name
    GAA (GAA Products)
    Synonyms
    LYAG antibody, E430018M07Rik antibody, glucosidase alpha, acid antibody, glucosidase, alpha, acid antibody, transmembrane and coiled-coil domain family 1 antibody, glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II) antibody, GAA antibody, Gaa antibody, TMCC1 antibody, gaa antibody
    Background
    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.
    Molecular Weight
    105324
    Gene ID
    2548
    NCBI Accession
    NP_000143, NP_001073271, NP_001073272
    UniProt
    P10253
    Pathways
    Cellular Glucan Metabolic Process
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