biological source
rabbit
Quality Segment
conjugate
unconjugated
material
colorless
clone
7G8T9, monoclonal
form
liquid
mol wt
50 kDa
species reactivity
human, rat
concentration
1.23mg/mL
technique(s)
western blot: 1:500 - 1:2000
color
colorless
isotype
IgG
immunogen sequence
FMCNLDCQEEPDSCISEKLFMEMAELMVSEGWKDAGYEYLCIDDCWMAPQRDSEGRLQADPQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFG
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... GLA(2717)
General description
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. [provided by RefSeq, Jul 2008]
Immunogen
A synthetic peptide corresponding to a sequence within amino acids 50-150 of human Galactosidase alpha (GLA) (GLA) (P06280).
PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.
Application
WB
存储类别
10 - Combustible liquids
wgk
WGK 1