Anti-Beta-glucocerebrosidase (GBA)

产品说明

一般描述

Glucosylceramidase (EC 3.2.1.45; UniProt P04062; also known as Acid beta-glucosidase, Alglucerase, Beta-GC, Beta-glucocerebrosidase, D-glucosyl-N-acylsphingosine glucohydrolase, Imiglucerase) is encoded by the GBA (also known as GC, GLUC) gene (Gene ID 2629) in human. It is a 62 kDa membrane associated lysosomal enzyme that catalyzes the hydrolysis of glucocerebroside to glucose and ceramide. It requires a signal peptide for transport across the membrane of the rough endoplasmic reticulum and glycosylation for transport into lysosomes where it is activated by saposin C. Over 300 different mutations have been identified in the GBA gene that lead to the deficiency or complete absence of glucosylceramidase activity leading to Gaucher s disease, one of the most common inherited disorder involving accumulation of glycolipids in macrophages, mainly in liver, spleen, and bone marrow. This disease is characterized by enlargement of liver and spleen, liver malfunction, anemia, bone lesions, and severe neurologic complications. Ref.: Novo JB et al. (2010). Mol. Biotechnol. 46, 279-286.

特异性

This mouse polyclonal antiserum detected the expected target band only in lysate from human beta-glucocerebrosidase-/GBA-transfected, but not untransfected, COS-7 cells. This polyclonal antiserum is reactive toward unglycosylated, glycosylated, as well as deglycosylated GBA following PNGase F treatment (Novo, J.B., et al. (2010). Mol. Biotechnol. 46(3):279-286).

免疫原

His-tagged recombinant full-length human mature Beta-glucocerebrosidase (GBA).

应用

Immunohistochemistry Analysis: A 1:250-1,000 dilution from a representative lot detected beta-glucocerebrosidase (GBA) in human cerebellum, cerebral cortex, and small intestine tissue sections.

Western Blotting Analysis: A representative lot detected His-tagged recombinant full-length human mature beta-glucocerebrosidase (GBA) (Courtesy of Dr. Cláudia Sossai Soares and Paulo Lee Ho).

ELISA Analysis: A representative lot detected purified recombinant human beta-glucocerebrosidase (GBA) (Novo, J.B., et al. (2010). Mol. Biotechnol. 46(3):279-286).

Western Blotting Analysis: A representative lot detected bacterially expressed unglycosylated recombinant human beta-glucocerebrosidase (GBA), glycosylated human GBA expressed and secreted by COS-7 transfectants, as well as endogenous GBA in HeLa and ECV304 cell lysates (Novo, J.B., et al. (2010). Mol. Biotechnol. 46(3):279-286).
Anti-Beta-glucocerebrosidase (GBA), Cat. No. ABN1387, is a highly specific mouse polyclonal antibody, that targets Glucosylceramidase and has been tested in ELISA, Immunohistochemistry (Paraffin), and Western Blotting.

质量

Evaluated by Western Blotting in SK-N-SH cell lysate.

Western Blotting Analysis: A 1,000 dilution of this antiserum detected beta-glucocerebrosidase (GBA) in 10 µg of SK-N-SH human neuroblastoma cell lysate.

目标描述

~59 kDa observed. 59.72/55.60 kDa (pro-/mature isoform Long), 57.46/55.60 kDa (pro-/mature isoform Short), 29.90 kDa (isoform 3) calculated. Uncharacterized bands may be observed in some lysate(s).

外形

Unpurified.
Mouse polyclonal antibody serum with 0.05% sodium azide.

其他说明

Concentration: Please refer to lot specific datasheet.

免责声明

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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