AB55080

Anti-GBA 抗体 [2E2]

Name: GBA抗体[2E2] (ab55080)| Abcam中文官网
Brand: Abcam Limited
SKU: AB55080
Price: 5135 CNY
Availability: InStock
Rating: 4 (3 reviews)

Anti-GBA antibody [2E2]

KO Validated
了解详情

(3 Reviews)

(22 Publications)

Anti-GBA antibody [2E2] (ab55080) is a mouse monoclonal antibody detecting GBA in Western Blot, IHC-P. Suitable for Human.

- KO validated for confirmed specificity
- Over 10 publications
- Trusted since 2007

查看别名

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

5 Images

Lab

Western blot - Anti-GBA antibody [2E2] (AB55080)

Lane 1 : Wild-type HAP1 whole cell lysate (40 μg)
Lane 2 : GBA knockout HAP1 whole cell lysate (40 μg)
Lane 3 : MCF7 whole cell lysate (40 μg)
Lane 4 : HepG2 whole cell lysate (40 μg)

Lanes 1 - 4 : Merged signal (red and green). Green - ab55080 observed at 70 kDa. Red - loading control, ab181602, observed at 37 kDa.

ab55080 was shown to specifically react with GBA in wild-type HAP1 cells along with additional cross-reactive bands. No bands were observed when GBA knockout samples were used. Wild-type and GBA knockout samples were subjected to SDS-PAGE. ab55080 and ab181602 (Rabbit anti GAPDH loading control) were incubated overnight at 4°C at 1 ug/ml and 1/10,000 dilution respectively. Blots were developed with Goat anti-Mouse IgG H&L (IRDye^® 800CW) preabsorbed (ab216772) and Goat anti-Rabbit IgG H&L (IRDye^® 680RD) preabsorbed (ab216777) secondary antibodies at 1/10,000 dilution for 1 hour at room temperature before imaging.

This image was generated using the ascites version of the product.

All lanes:

Western blot - Anti-GBA antibody [2E2] (ab55080)

Predicted band size: 60 kDa

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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-GBA antibody [2E2] (AB55080)

IHC-P

Unknown

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-GBA antibody [2E2] (AB55080)

GBA antibody (ab55080) used in immunohistochemistry at 3ug/ml on formalin fixed and paraffin embedded human breast cancer.

This image was generated using the ascites version of the product.

Unknown

Western blot - Anti-GBA antibody [2E2] (AB55080)

GBA antibody (ab55080) at 1ug/lane + MCF-7 cell lysate at 25ug/lane.

This image was generated using the ascites version of the product.

All lanes:

Western blot - Anti-GBA antibody [2E2] (ab55080)

Predicted band size: 60 kDa

false

Supplier Data

Western blot - Anti-GBA antibody [2E2] (AB55080)

ab55080 was shown to react with GBA1 in wild-type HAP1 cells in Western blot with loss of signal observed in a GBA1 knockout cell line. Wild-type HAP1 and GBA1 knockout cell lysates were subjected to SDS-PAGE. Membranes were blocked in 5% milk in TBST for 1 hr before incubation with ab55080 overnight at 4 °C at a 1/500 dilution. Blots were incubated with secondary antibodies at 0.2 µg/mL before imaging.

This data was provided by YCharOS Inc., an open science company with the mission of characterizing commercially available antibody reagents for all human proteins. Abcam and YCharOS are working together to help address the reproducibility crisis by enabling the life science community to better evaluate commercially available antibodies.

All lanes:

Western blot - Anti-GBA antibody [2E2] (ab55080) at 1/500 dilution

Lane 1:

Wild-type HAP1 lysate at 30 µg

Lane 2:

GBA1 knock-out HAP1 lysate at 30 µg

false

AbReview14732****

Western blot - Anti-GBA antibody [2E2] (AB55080)

This image was generated using the ascites version of the product.

All lanes:

Western blot - Anti-GBA antibody [2E2] (ab55080) at 1/1000 dilution

Lane 1:

Lysate prepared from MOCK at 10 µg

Lane 2:

Lysate prepared from human HN10 cells at 10 µg

Secondary

All lanes:

IRDye® donkey polyclonal to mouse IgG at 1/3000 dilution

Predicted band size: 60 kDa

Observed band size: 60 kDa

false

Exposure time: 1min

This image is a courtesy of Anonymous Abreview

关键信息

宿主种属

Mouse

克隆

Monoclonal

克隆号

2E2

亚型

IgG2a

轻链类型

kappa

不含载体蛋白

反应种属

Human

应用

IHC-P, WB

applications

免疫原

Recombinant Fragment Protein within Human GBA1 aa 100-250. The exact immunogen used to generate this antibody is proprietary information.

P04062

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反应性数据

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"}, "IHCP" : {"fullname" : "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)", "shortname":"IHC-P"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "", "WB-species-notes": "<p></p>", "IHCP-species-checked": "testedAndGuaranteed", "IHCP-species-dilution-info": "", "IHCP-species-notes": "<p></p>" } } }

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产品详情

This product was changed from ascites to tissue culture supernatant on 15 May 2019. Please note that the dilutions may need to be adjusted accordingly. If you have any questions, please do not hesitate to contact our scientific support team.

Product Specifications
Anti-GBA antibody [2E2] (ab55080) is a mouse monoclonal antibody and is validated for use in ICC/IF, IHC-P, WB in human samples.
Anti-GBA antibody [2E2] (ab55080) specifically detects GBA (UniProt ID: P04062; Molecular weight: 56kDa) and is sold in 100 µg selling sizes.

Quality and Validation
Abcam's high quality validation processes ensure Anti-GBA antibody [2E2] (ab55080) has high sensitivity and specificity.
The specificity of Anti-GBA antibody [2E2] (ab55080) has been confirmed by testing in knockout samples.
Anti-GBA antibody [2E2] (ab55080) has been cited over 14 times in peer reviewed journals and is trusted by the scientific community.

Related Products
Antibody clone 2E2 is also available pre-conjugated to a variety of labels for your convenience - PE (ab95673).

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性能和储存信息

形式

Liquid

纯化工艺

Affinity purification Protein A

存储溶液

pH: 7.4

运输条件

Blue Ice

分装信息

Upon delivery aliquot

储存信息

Avoid freeze / thaw cycle

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补充信息

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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产品实验方案

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

Visit the General protocols
Visit the Troubleshooting

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靶点信息

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 : 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). 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).

See full target information GBA1

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文献 (22)

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

Drug delivery and translational research : PubMed40836186

2025

Polymeric nanoparticle-mediated GBA1 gene therapy is neuroprotective in a preclinical model of Parkinson's disease.

Applications

Unspecified application

Species

Unspecified reactive species

Mohit Kwatra,Gijung Kwak,Haolin Li,Jung Soo Suk,Han Seok Ko

NPJ Parkinson's disease 10:142 PubMed39095359

2024

GBA-AAV mitigates sleep disruptions and motor deficits in mice with REM sleep behavior disorder.

Applications

Unspecified application

Species

Unspecified reactive species

Ying Chen,Wei-Ye Xie,Dong Xia,Mu-Tian Zhang,Yan-Rui Sun,Wen-Xiang Duan,Yun Shen,Fen Wang,Wei-Min Qu,Zhi-Li Huang,Chun-Feng Liu

Science advances 10:eadk1296 PubMed38924406

2024

The annotation of has been concealed by its protein-coding pseudogene .

Applications

Unspecified application

Species

Unspecified reactive species

Emil K Gustavsson,Siddharth Sethi,Yujing Gao,Jonathan W Brenton,Sonia García-Ruiz,David Zhang,Raquel Garza,Regina H Reynolds,James R Evans,Zhongbo Chen,Melissa Grant-Peters,Hannah Macpherson,Kylie Montgomery,Rhys Dore,Anna I Wernick,Charles Arber,Selina Wray,Sonia Gandhi,Julian Esselborn,Cornelis Blauwendraat,Christopher H Douse,Anita Adami,Diahann A M Atacho,Antonina Kouli,Annelies Quaegebeur,Roger A Barker,Elisabet Englund,Frances Platt,Johan Jakobsson,Nicholas W Wood,Henry Houlden,Harpreet Saini,Carla F Bento,John Hardy,Mina Ryten

Scientific reports 14:9177 PubMed38649404

2024

An increase in ER stress and unfolded protein response in iPSCs-derived neuronal cells from neuronopathic Gaucher disease patients.

Applications

Unspecified application

Species

Unspecified reactive species

Tanapat Pornsukjantra,Nongluk Saikachain,Nareerat Sutjarit,Arthaporn Khongkrapan,Alisa Tubsuwan,Kanit Bhukhai,Thipwimol Tim-Aroon,Usanarat Anurathapan,Suradej Hongeng,Nithi Asavapanumas

Biological & pharmaceutical bulletin 47:827-839 PubMed38599826

2024

Protective Effects of Coptis chinensis Rhizome Extract and Its Constituents (Berberine, Coptisine, and Palmatine) against α-Synuclein Neurotoxicity in Dopaminergic SH-SY5Y Cells.

Applications

Unspecified application

Species

Unspecified reactive species

Chih-Hsin Lin,Yih-Ru Wu,Chih-Ying Chao,Kuo-Hsuan Chang,Chiung-Mei Chen,Wan-Ling Chen,Pei-Ning Yang,Guey-Jen Lee-Chen

Journal of Parkinson's disease 14:65-78 PubMed38251062

2024

Characterization of Novel Human β-glucocerebrosidase Antibodies for Parkinson's Disease Research.

Applications

Unspecified application

Species

Unspecified reactive species

Tiffany Jong,Alexandra Gehrlein,Ellen Sidransky,Ravi Jagasia,Yu Chen

NPJ Parkinson's disease 8:126 PubMed36202848

2022

Lysosomal lipid alterations caused by glucocerebrosidase deficiency promote lysosomal dysfunction, chaperone-mediated-autophagy deficiency, and alpha-synuclein pathology.

Applications

Unspecified application

Species

Unspecified reactive species

Alba Navarro-Romero,Irene Fernandez-Gonzalez,Jordi Riera,Marta Montpeyo,Merce Albert-Bayo,Tresa Lopez-Royo,Pablo Castillo-Sanchez,Clara Carnicer-Caceres,Jose Antonio Arranz-Amo,Laura Castillo-Ribelles,Eddie Pradas,Josefina Casas,Miquel Vila,Marta Martinez-Vicente

Brain : a journal of neurology 145:1038-1051 PubMed35362022

2022

Sphingolipid changes in Parkinson L444P GBA mutation fibroblasts promote α-synuclein aggregation.

Applications

Unspecified application

Species

Unspecified reactive species

Céline Galvagnion,Frederik Ravnkilde Marlet,Silvia Cerri,Anthony H V Schapira,Fabio Blandini,Donato A Di Monte

Proceedings of the National Academy of Sciences of the United States of America 118: PubMed34326260

2021

Wild-type GBA1 increases the α-synuclein tetramer-monomer ratio, reduces lipid-rich aggregates, and attenuates motor and cognitive deficits in mice.

Applications

Unspecified application

Species

Unspecified reactive species

Kelly E Glajch,Tim E Moors,Yi Chen,Pascal A Bechade,Alice Y Nam,Molly M Rajsombath,Thomas D McCaffery,Ulf Dettmer,Andreas Weihofen,Warren D Hirst,Dennis J Selkoe,Silke Nuber

International journal of molecular sciences 22: PubMed34062940

2021

Glucocerebrosidase Gene Therapy Induces Alpha-Synuclein Clearance and Neuroprotection of Midbrain Dopaminergic Neurons in Mice and Macaques.

Applications

Unspecified application

Species

Unspecified reactive species

Diego Sucunza,Alberto J Rico,Elvira Roda,María Collantes,Gloria González-Aseguinolaza,Ana I Rodríguez-Pérez,Iván Peñuelas,Alfonso Vázquez,José L Labandeira-García,Vania Broccoli,José L Lanciego

View all publications

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