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  • ABN1387 - Anti-Beta-glucocerebrosidase (GBA)

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ABN1387 Sigma-Aldrich

Anti-Beta-glucocerebrosidase (GBA)

serum, clone, from mouse

Synonym: Glucosylceramidase, Acid beta-glucosidase, Alglucerase, Beta-GC, Beta-glucocerebrosidase, D-glucosyl-N-acylsphingosine glucohydrolase, Imiglucerase

  •  eCl@ss 32160702

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Properties

Related Categories Alphabetical Index, Antibodies, B-BI, Primary Antibodies
clone   polyclonal
biological source   mouse
application(s)   ELISA: suitable
  immunohistochemistry: suitable (paraffin)
  western blot: suitable
species reactivity   human
shipped in   ambient
antibody form   serum
Quality Level   100
antibody product type   primary antibodies
NCBI accession no.   NP_000148
UniProt accession no.   P04062
Gene Information   human ... GBA(2629)

Description

General description

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.

Specificity

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).

Immunogen

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

Application

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.

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).

Target description

~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).

Physical form

Mouse polyclonal antibody serum with 0.05% sodium azide.

Quality

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.

Other Notes

Concentration: Please refer to lot specific datasheet.

Safety & Documentation

Safety Information

WGK Germany 
WGK 1
Flash Point(F) 
Not applicable
Flash Point(C) 
Not applicable
Protocols & Articles

Articles

Antibody Basics

Immunoglobulins (Igs) are produced by B lymphocytes and secreted into plasma. The Ig molecule in monomeric form is a glycoprotein with a molecular weight of approximately 150 kDa that is shaped more ...
Keywords: Affinity chromatography, Centrifugation, Chromatography, Digestions, Direct immunofluorescence, Gene expression, High performance liquid chromatography, Immunofluorescence, Ion Exchange, Microscopy, Precipitation, Purification, Rheumatology, Scanning electron microscopy

Protocols

Western Blot Protocol | Immunoblotting Protocol

Western Blotting refers to the electrophoretic transfer of proteins from sodium dodecyl sulfate polyacrylamide gels to sheets of PVDF or nitrocellullose membrane, followed by immunodetection of prote...
Keywords: AGE, Buffers, Cell disruption, Detection methods, Detergents, Dialysis, Electroblotting, Electrophoresis, Enzyme activity, Gel electrophoresis, Immunoprecipitation, PAGE, Protein extraction, Purification, Sample preparations, Western blot

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