GAA Rabbit Polyclonal Antibody

CAT#: TA376424

GAA Rabbit polyclonal Antibody

Specifications

Product Data

• Applications: IF, IP, WB
• Recommended Dilution: WB,1:500 - 1:2000
  IF,1:50 - 1:200
  IP,1:50 - 1:200
• Reactivities: Human, Mouse, Rat
• Modifications: Unmodified
• Host: Rabbit
• Isotype: IgG
• Clonality: Polyclonal
• Immunogen: A synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2).
• Formulation: Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
• Concentration: lot specific
• Purification: Affinity purification
• Conjugation: Unconjugated
• Stability: Shelf life: one year from despatch.
• Predicted Protein Size: 105kDa
• Gene Name: glucosidase alpha, acid
• Database Link:
  Entrez Gene 2548 Human
  UniProt ID P10253
• Background: This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants.