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生物源
rabbit
品質等級
共軛
unconjugated
抗體表格
affinity isolated antibody
抗體產品種類
primary antibodies
無性繁殖
polyclonal
形狀
buffered aqueous solution
分子量
45 kDa
物種活性
rat, mouse, guinea pig, human, dog
濃度
0.5 mg - 1 mg/mL
技術
western blot: suitable
NCBI登錄號
UniProt登錄號
運輸包裝
wet ice
儲存溫度
−20°C
目標翻譯後修改
unmodified
基因資訊
human ... GLA(2717)
免疫原
Synthetic peptide directed towards the N terminal region of human GLA
應用
Anti-GLA antibody produced in rabbit is suitable for western blotting at a concentration of 1μg/mL.
生化／生理作用
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.
序列
Synthetic peptide located within the following region: PQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTF
外觀
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
免責聲明
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.
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儲存類別代碼
10 - Combustible liquids
水污染物質分類（WGK）
WGK 3
閃點（°F）
Not applicable
閃點（°C）
Not applicable
D P Germain et al.
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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