Galactosidase alpha (GLA) Rabbit Polyclonal Antibody

CAT#: TA376586

Galactosidase alpha (GLA) Rabbit polyclonal Antibody

Specifications

Product Data

• Applications: WB
• Recommended Dilution: WB,1:500 - 1:2000
• Reactivities: Human
• Modifications: Unmodified
• Host: Rabbit
• Isotype: IgG
• Clonality: Polyclonal
• Immunogen: Recombinant fusion protein containing a sequence corresponding to amino acids 150-429 of human Galactosidase alpha (Galactosidase alpha (GLA)) (NP_000160.1).
• 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: 48kDa
• Gene Name: galactosidase alpha
• Database Link:
  Entrez Gene 2717 Human
  UniProt ID P06280
• Background: This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties.