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Galactosidase alpha antibody, α-半乳糖苷酶抗体

 
编 号 PR-4581
产品名称 Galactosidase alpha antibody, α-半乳糖苷酶抗体
规 格 0.2ml
价 格 1980元
应 用 IHC,WB,ELISA,ICC,IP,IF
品 牌 Hopebiot

详细信息: 说明书下载

Background:  Galactosidase alpha is involved in the hydrolysis of terminal, non reducing alpha D galactose residues in alpha D galactosides, including galactose oligosaccharides, galactomannans and galactohydrolase. Defects in GLA are the cause of Fabry's disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.

Description: Rabbit polyclonal to Galactosidase alpha

Immunogen: KLH conjugated synthetic peptide derived from Galactosidase alpha

Specificity:  ·Reacts with Human, Mouse, Pig, Dog and Rat.

·Isotype: IgG

Application:  ·Western blotting: 1/100-500. Predicted Mol wt: 32 kDa;

·Immunohistochemistry (Paraffin/frozen tissue section): 1/50-200;

·Immunocytochemistry/Immunofluorescence: 1/100;

·Immunoprecipitation: 1/50;

·ELISA: 1/500;

·Optimal working dilutions must be determined by the end user.

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