Galactosidase alpha (GLA) Rabbit Polyclonal Antibody
CNY 5,250.00
货期*
6周
规格
Cited in 1 publication. |
经常一起买 (4)
beta Actin Mouse Monoclonal Antibody, Clone OTI1, Loading Control
CNY 300.00
CNY 1,430.00
Specifications
Product Data | |
Applications | WB |
Recommend Dilution | WB |
Reactivity | Human |
Host | Rabbit |
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. |
Purification | Affinity Purified |
Conjugation | Unconjugated |
Storage Condition | Store at -20°C as received. |
Predicted Protein Size | 45 kDa |
Gene Name | galactosidase alpha |
Database Link | |
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 |
Citations (1)
The use of this Antibodies has been cited in the following citations: |
---|
Accumulation of α -synuclein mediates podocyte injury in Fabry nephropathy
,null,
The Journal of Clinical Investigation
,PubMed ID 37014703
[GLA]
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