Galactosidase alpha (GLA) (N-term) Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IHC, WB
Recommend Dilution	Western blot: 1/50 - 1/100. ELISA: 1/1,000. Immunohistochemistry on paraffin sections: 1/50 - 1/100.
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Immunogen	KLH conjugated synthetic peptide between 90~120 amino acids from the N-terminal region of human GLA
Specificity	This antibody detects GLA at N-term.
Formulation	PBS with 0.09% (W/V) sodium azide as preservative State: Liquid purified Ig fraction
Concentration	lot specific
Purification	Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS
Conjugation	Unconjugated
Storage Condition	Store the antibody at 2 - 8 °C up to one month or (in aliquots) at -20 °C for longer. Avoid repeated freezing and thawing.
Gene Name	galactosidase alpha
Database Link	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.
Synonyms	Alpha-D-galactosidase A, Melibiase
Note	Molecular weight: 48767 Da
Reference Data
Protein Families	Druggable Genome
Protein Pathways	Galactose metabolism, Glycerolipid metabolism, Glycosphingolipid biosynthesis - globo series, Lysosome, Sphingolipid metabolism

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