GAA (N-term) Rabbit Polyclonal Antibody

CAT#: AP51752PU-N

GAA (N-term) rabbit polyclonal antibody, Aff - Purified

Product Images

Western blot analysis of Alpha-glucosidase Antibody (N-term) in HL-60 cell line lysates (35ug/lane).

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  Immunohistochemistry analysis in formalin fixed and paraffin embedded human lung carcinoma reacted with Alpha-glucosidase Antibody (N-term) followed by peroxidase conjugation of the secondary antibody and DAB staining.

Specifications

Product Data

• Applications: IHC, WB
• Recommended Dilution: ELISA: 1/1000.
  Western Blot: 1/100-1/500.
  Immunohistochemistry on Paraffin Sections: 1/10-1/50.
• Reactivities: Human
• Host: Rabbit
• Isotype: Ig
• Clonality: Polyclonal
• Immunogen: conjugated synthetic peptide between 173-203 amino acids from the N-terminal region of Human Alpha-glucosidase
• Specificity: This antibody recognizes Human Alpha-glucosidase (N-term).
• Formulation: PBS containing 0.09% (W/V) Sodium Azide as preservative
  State: Aff - Purified
  State: Liquid purified Ig fraction
• Concentration: lot specific
• Purification: Protein A column, followed by peptide affinity purification
• Storage:

  Store undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer.
  Avoid repeated freezing and thawing.

• Stability: Shelf life: one year from despatch.

• Gene Name: Homo sapiens glucosidase, alpha; acid (GAA), transcript variant 1
• Database Link:
  Entrez Gene 2548 Human
• 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.
• Synonyms: Acid maltase, Aglucosidase alfa, GAA
• Note: Molecular Weight: 105319 Da

Reference Data

• Protein Families: Druggable Genome, Transmembrane
• Protein Pathways: Galactose metabolism, Lysosome, Metabolic pathways, Starch and sucrose metabolism