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AB96246

Anti-GBA antibody

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(3 Publications)

Rabbit Polyclonal GBA antibody. Suitable for WB, IHC-P, ICC/IF and reacts with Human samples. Cited in 3 publications. Immunogen corresponding to Recombinant Fragment Protein within Human GBA1 aa 350 to C-terminus.

View Alternative Names

GBA, GC, GLUC, GBA1, Lysosomal acid glucosylceramidase, Lysosomal acid GCase, Acid beta-glucosidase, Alglucerase, Beta-glucocerebrosidase, Beta-glucosylceramidase 1, Cholesterol glucosyltransferase, Cholesteryl-beta-glucosidase, D-glucosyl-N-acylsphingosine glucohydrolase, Glucosylceramidase beta 1, Imiglucerase, Lysosomal cholesterol glycosyltransferase, Lysosomal galactosylceramidase, Lysosomal glycosylceramidase, Beta-GC, SGTase

3 Images
Immunocytochemistry/ Immunofluorescence - Anti-GBA antibody (AB96246)
  • ICC/IF

Supplier Data

Immunocytochemistry/ Immunofluorescence - Anti-GBA antibody (AB96246)

Immunofluorescence analysis of methanol-fixed HeLa, using ab96246 at 1 : 100 dilution. Counterstained with Hoechst 33342 (blue)

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-GBA antibody (AB96246)
  • IHC-P

Supplier Data

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-GBA antibody (AB96246)

Immunohistochemical analysis of paraffin-embedded human lung cancer, using ab96246 at 1 : 100 dilution.

Western blot - Anti-GBA antibody (AB96246)
  • WB

Unknown

Western blot - Anti-GBA antibody (AB96246)

7.5% SDS PAGE

All lanes:

Western blot - Anti-GBA antibody (ab96246) at 1/500 dilution

All lanes:

HepG2 whole cell lysate at 30 µg

Predicted band size: 60 kDa

false

Key facts

Host species

Rabbit

Clonality

Polyclonal

Isotype

IgG

Carrier free

No

Reacts with

Human

Applications

IHC-P, ICC/IF, WB

applications

Immunogen

Recombinant Fragment Protein within Human GBA1 aa 350 to C-terminus. The exact immunogen used to generate this antibody is proprietary information.

P04062

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Reactivity data

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Properties and storage information

Form
Liquid
Purification technique
Affinity purification Immunogen
Storage buffer
pH: 7 Preservative: 0.025% Proclin 300 Constituents: PBS, 20% Glycerol (glycerin, glycerine)
Shipped at conditions
Blue Ice
Appropriate short-term storage conditions
+4°C
Appropriate long-term storage conditions
-20°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle
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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

GBA also known as glucosylceramidase is a lysosomal enzyme with a molecular mass of approximately 59 kDa. This enzyme breaks down glucosylceramide into glucose and ceramide. GBA is expressed predominantly in tissues with high metabolic activities such as the brain liver and spleen. Its function relies on its catalytic activity where substrates bind to its active site enabling the hydrolysis process necessary for maintaining cellular metabolism.
Biological function summary

GBA plays an important role in sphingolipid metabolism. It participates in the degradation of glycolipids within the lysosome contributing to lipid recycling. It acts independently rather than as a part of a major enzymatic complex. Through its role in degrading glucosylceramide GBA influences cellular homeostasis and bioenergetics ensuring balance in neural and systemic lipid levels.

Pathways

GBA’s enzymatic functions are integral to the glycosphingolipid metabolic pathway. It is involved in the downstream steps of the lysosomal degradation of glycosphingolipids. The pathway operates alongside other important proteins such as beta-glucosidase and CERT-related transfer proteins all of which contribute to membrane lipid organization and signal transduction processes.

GBA mutations are linked with Gaucher disease and Parkinson’s disease. In Gaucher disease deficient GBA activity leads to substrate accumulation resulting in hepatosplenomegaly and other systemic symptoms. Reduced GBA activity is also associated with increased alpha-synuclein aggregation in Parkinson’s disease implicating it in the pathogenesis of neurodegenerative disorders. The enzyme’s function in these diseases highlights its role in maintaining cellular equilibrium and signaling pathways.
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Product protocols

For this product, it's our understanding that no specific protocols are required. You can visit:
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Target data

Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramides/GlcCers (such as beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine) into free ceramides (such as N-acylsphing-4-enine) and glucose (PubMed : 15916907, PubMed : 24211208, PubMed : 32144204, PubMed : 39395789, PubMed : 9201993). Plays a central role in the degradation of complex lipids and the turnover of cellular membranes (PubMed : 27378698). Through the production of ceramides, participates in the PKC-activated salvage pathway of ceramide formation (PubMed : 19279011). Catalyzes the glucosylation of cholesterol, through a transglucosylation reaction where glucose is transferred from GlcCer to cholesterol (PubMed : 24211208, PubMed : 26724485, PubMed : 32144204). GlcCer containing mono-unsaturated fatty acids (such as beta-D-glucosyl-N-(9Z-octadecenoyl)-sphing-4-enine) are preferred as glucose donors for cholesterol glucosylation when compared with GlcCer containing same chain length of saturated fatty acids (such as beta-D-glucosyl-N-octadecanoyl-sphing-4-enine) (PubMed : 24211208). Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl 3-beta-D-glucoside to ceramide (Probable) (PubMed : 26724485). Can also hydrolyze cholesteryl 3-beta-D-glucoside producing glucose and cholesterol (PubMed : 24211208, PubMed : 26724485, PubMed : 39395789). Catalyzes the hydrolysis of galactosylceramides/GalCers (such as beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine), as well as the transfer of galactose between GalCers and cholesterol in vitro, but with lower activity than with GlcCers (PubMed : 32144204). Contrary to GlcCer and GalCer, xylosylceramide/XylCer (such as beta-D-xyosyl-(1<->1')-N-acylsphing-4-enine) is not a good substrate for hydrolysis, however it is a good xylose donor for transxylosylation activity to form cholesteryl 3-beta-D-xyloside (PubMed : 33361282). Can also metabolize plant glycosyl phytosterols such as glucosylstigmasterol (PubMed : 39395789).
See full target information GBA1
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Publications (3)

Recent publications for all applications. Explore the full list and refine your search

Science advances 9:eadf2746 PubMed37390205

2023

A localized hydrogel-mediated chemotherapy causes immunogenic cell death via activation of ceramide-mediated unfolded protein response.

Applications

Unspecified application

Species

Unspecified reactive species

Animesh Kar,Dolly Jain,Sandeep Kumar,Kajal Rajput,Sanjay Pal,Kajal Rana,Raunak Kar,Somesh K Jha,Nihal Medatwal,Prabhu Srinivas Yavvari,Nishant Pandey,Devashish Mehta,Harsh Sharma,Debanjan Bhattacharya,Manas K Pradhan,Ravi Datta Sharma,Aasheesh Srivastava,Usha Agrawal,Arnab Mukhopadhyay,Sagar Sengupta,Veena S Patil,Avinash Bajaj,Ujjaini Dasgupta

The Journal of clinical investigation 133: PubMed37014703

2023

Accumulation of α-synuclein mediates podocyte injury in Fabry nephropathy.

Applications

Unspecified application

Species

Unspecified reactive species

Fabian Braun,Ahmed Abed,Dominik Sellung,Manuel Rogg,Mathias Woidy,Oysten Eikrem,Nicola Wanner,Jessica Gambardella,Sandra D Laufer,Fabian Haas,Milagros N Wong,Bernhard Dumoulin,Paula Rischke,Anne Mühlig,Wiebke Sachs,Katharina von Cossel,Kristina Schulz,Nicole Muschol,Sören W Gersting,Ania C Muntau,Oliver Kretz,Oliver Hahn,Markus M Rinschen,Michael Mauer,Tillmann Bork,Florian Grahammer,Wei Liang,Thorsten Eierhoff,Winfried Römer,Arne Hansen,Catherine Meyer-Schwesinger,Guido Iaccarino,Camilla Tøndel,Hans-Peter Marti,Behzad Najafian,Victor G Puelles,Christoph Schell,Tobias B Huber

Molecular cancer research : MCR 17:1893-1909 PubMed31239287

2019

Cigarette Smoke Induces Metabolic Reprogramming of the Tumor Stroma in Head and Neck Squamous Cell Carcinoma.

Applications

Unspecified application

Species

Unspecified reactive species

Marina Domingo-Vidal,Diana Whitaker-Menezes,Cristina Martos-Rus,Patrick Tassone,Christopher M Snyder,Madalina Tuluc,Nancy Philp,Joseph Curry,Ubaldo Martinez-Outschoorn
View all publications
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Product promise

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