Galactosidase alpha Recombinant Rabbit mAb (一抗) - WB,IHC-P,IHC-F,IF,IP,Flow-Cyt | Bioss

bsm-62171R

重组兔单抗、mIHC精品抗体

Galactosidase alpha Recombinant Rabbit mAb

α-半乳糖苷酶重组兔单抗

GALA; Ags; AGAL_HUMAN; GLA; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Galactosylgalactosylglucosylceramidase GLA; Melibiase; 3.2.1.22; AGAL_MOUSE; galactosidase alpha

Rabbit

A synthesized peptide derived from human alpha Galactosidase A: 100-150/429

IgG

Liquid

affinity purified by Protein A

Recombinant

4D11

49 kDa

47 kDa

10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% Proclin300 and 50% glycerol.

Store at 4℃ for short term. Store at -20℃ for long term. Avoid repeated freeze/thaw cycles.

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

Catalyzes the hydrolysis of glycosphingolipids and participates in their degradation in the lysosome.

GLA

alpha-galactosidase A

Homodimer.

Lysosome.

Defects in GLA are the cause of Fabry disease (FD) [MIM:301500]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. 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.

Belongs to the glycosyl hydrolase 27 family.