Galactosidase alpha (GLA) Rabbit Polyclonal Antibody

CAT#: TA336243

Rabbit Polyclonal Anti-GLA Antibody

Product Images

WB Suggested Anti-GLA Antibody Titration: 0.2-1 ug/ml; Positive Control: Human brain

Specifications

Product Data

• Applications: WB
• Recommended Dilution: WB
• Reactivities: Human
• Host: Rabbit
• Isotype: IgG
• Clonality: Polyclonal
• Immunogen: The immunogen for Anti-GLA Antibody: synthetic peptide directed towards the N terminal of human GLA. Synthetic peptide located within the following region: PQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTF
• Formulation: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
  Note that this product is shipped as lyophilized powder to China customers.
• Purification: Affinity Purified
• Conjugation: Unconjugated
• Storage: Store at -20°C as received.
• Stability: Stable for 12 months from date of receipt.
• Predicted Protein Size: 45 kDa
• Gene Name: galactosidase alpha
• Database Link:
  NP_000160
  Entrez Gene 2717 Human
  UniProt ID P06280
• Background: GLA is 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.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. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.
• Synonyms: GALA
• Note: Immunogen Sequence Homology: Dog: 100%; Pig: 100%; Rat: 100%; Human: 100%; Mouse: 100%; Guinea pig: 100%; Bovine: 93%; Rabbit: 93%; Zebrafish: 93%; Goat: 86%; Horse: 86%

Reference Data

• Protein Families: Druggable Genome
• Protein Pathways: Galactose metabolism, Glycerolipid metabolism, Glycosphingolipid biosynthesis - globo series, Lysosome, Sphingolipid metabolism