Synthetic peptide directed towards the N terminal region of human GLA
Application
Anti-GLA antibody produced in rabbit is suitable for western blotting at a concentration of 1μg/mL.
Biochem/physiol Actions
GLA gene encodes a homodimeric glycoprotein that hydrolyzes the terminal α-galactosyl moieties from glycolipids and glycoproteins. It also plays a role in catalysing hydrolysis of melibiose into galactose and glucose. Mutation in GLA gene results in fabry disease, a rare lysosomal storage disorder associated with failure to catabolize α-D-galactosyl glycolipid moieties.
Sequence
Synthetic peptide located within the following region: PQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTF
Physical form
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Biochemical and biophysical research communications, 257(3), 708-713 (1999-04-20)
Fabry disease (FD) (angiokeratoma corporis diffusum) is an X-linked inborn error of glycosphingolipid metabolism caused by defects in the lysosomal alpha-galactosidase A gene (GLA). The enzymatic defect leads to the systemic accumulation of neutral glycosphingolipids with terminal alpha-galactosyl moieties. Clinically
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