GAA Antibody / Glucosidase alpha acid

• Description:

  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.

• UniProt:

  P10253

• Host:

  Rabbit

• Immunogen:

  A portion of amino acids 174-203 from the human protein were used as the immunogen for the GAA antibody.

• Clonality:

  Polyclonal

• Isotype:

  Ig

• Applications:

  WB, IHC-P

• Format:

  Purified

• Buffer:

  In 1X PBS, pH 7.4, with 0.09% sodium azide

• Reconstitution:

  Aliquot the GAA antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.

• Limitations:

  This GAA antibody is available for research use only.

• CAS Number:

  9007-83-4