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Glucosidase, Alpha, Acid (GAA) Peptide

GAA Reactivity: Human Host: Synthetic BP, IHC, WB
Catalog No. ABIN977187
  • Target See all GAA products
    GAA (Glucosidase, Alpha, Acid (GAA))
    Origin
    Human
    Source
    • 1
    Synthetic
    Application
    Blocking Peptide (BP), Immunohistochemistry (IHC), Western Blotting (WB)
    Characteristics
    This is a synthetic peptide designed for use in combination with anti-GAA antibody (Catalog #: ARP44226_P050). It may block above mentioned antibody from binding to its target protein in western blot and/or immunohistochecmistry under proper experimental settings. There is no guarantee for its use in other applications.
    Purification
    Purified
  • Application Notes
    Each Investigator should determine their own optimal working dilution for specific applications.
    Restrictions
    For Research Use only
  • Format
    Lyophilized
    Reconstitution
    Add 100 μL of sterile PBS. Final peptide concentration is 1 mg/mL in PBS.
    Concentration
    1 mg/mL
    Buffer
    Final peptide concentration is 1 mg/mL in PBS.
    Handling Advice
    Avoid repeated freeze-thaw cycles.
    Storage
    -20 °C
    Storage Comment
    For longer periods of storage, store at -20°C. Avoid repeat freeze-thaw cycles.
  • Target
    GAA (Glucosidase, Alpha, Acid (GAA))
    Background
    GAA is 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. 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.

    Alias Symbols: LYAG

    Protein Interaction Partner: NCF1

    Protein Size: 952
    Molecular Weight
    98 kDa
    Gene ID
    2548
    NCBI Accession
    NM_000152, NP_000143
    UniProt
    P10253
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