AB180596

Anti-GBE1抗体[EP11113]

Anti-GBE1 antibody [EP11113]

RabMAb
了解详情

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(11 Publications)

Rabbit Monoclonal GBE1 antibody. Suitable for WB and reacts with Human samples. Cited in 11 publications.

查看别名

Brancher enzyme, Glycogen-branching enzyme, GBE1

2 Images

Supplier Data

Western blot - Anti-GBE1 antibody [EP11113] (AB180596)

All lanes:

Western blot - Anti-GBE1 antibody [EP11113] (ab180596) at 1/10000 dilution

Lane 1:

Human fetal liver lysate at 20 µg

Lane 2:

PC-3 lysate at 20 µg

Secondary

All lanes:

Goat Anti-Rabbit IgG (H+L) Peroxidase conjugated at 1/1000 dilution

Predicted band size: 80 kDa

false

Supplier Data

Western blot - Anti-GBE1 antibody [EP11113] (AB180596)

All lanes:

Western blot - Anti-GBE1 antibody [EP11113] (ab180596) at 1/2000 dilution

All lanes:

Human skeletal muscle lysate at 10 µg

Secondary

All lanes:

Western blot - Goat Anti-Rabbit IgG H&L (HRP) (<a href='/products/secondary-antibodies/goat-rabbit-igg-h-l-hrp-ab136636'>ab136636</a>) at 1/500 dilution

Predicted band size: 76 kDa,80 kDa

false

不同偶联物与剂型 (1)

Carrier free

Anti-GBE1 antibody [EP11113] - BSA and Azide free

关键信息

宿主种属

Rabbit

克隆

Monoclonal

克隆号

EP11113

亚型

IgG

不含载体蛋白

反应种属

Human

应用

applications

免疫原

The exact immunogen used to generate this antibody is proprietary information.

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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"}, "ICCIF" : {"fullname" : "Immunocytochemistry/ Immunofluorescence", "shortname":"ICC/IF"}, "FlowCytIntra" : {"fullname" : "Flow Cytometry (Intracellular)", "shortname":"Flow Cyt (Intra)"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/1000 - 1/10000", "WB-species-notes": "", "ICCIF-species-checked": "notRecommended", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "", "FlowCytIntra-species-checked": "notRecommended", "FlowCytIntra-species-dilution-info": "", "FlowCytIntra-species-notes": "" }, "Mouse": { "WB-species-checked": "predicted", "WB-species-dilution-info": "", "WB-species-notes": "", "ICCIF-species-checked": "notRecommended", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "", "FlowCytIntra-species-checked": "notRecommended", "FlowCytIntra-species-dilution-info": "", "FlowCytIntra-species-notes": "" }, "Rat": { "WB-species-checked": "predicted", "WB-species-dilution-info": "", "WB-species-notes": "", "ICCIF-species-checked": "notRecommended", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "", "FlowCytIntra-species-checked": "notRecommended", "FlowCytIntra-species-dilution-info": "", "FlowCytIntra-species-notes": "" }, "Cat": { "WB-species-checked": "predicted", "WB-species-dilution-info": "", "WB-species-notes": "", "ICCIF-species-checked": "notRecommended", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "", "FlowCytIntra-species-checked": "notRecommended", "FlowCytIntra-species-dilution-info": "", "FlowCytIntra-species-notes": "" }, "Horse": { "WB-species-checked": "predicted", "WB-species-dilution-info": "", "WB-species-notes": "", "ICCIF-species-checked": "notRecommended", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "", "FlowCytIntra-species-checked": "notRecommended", "FlowCytIntra-species-dilution-info": "", "FlowCytIntra-species-notes": "" } } }

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产品详情

Patented technology
Our RabMAb^® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb^® patents.

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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: 7.2 - 7.4 Preservative: 0.01% Sodium azide Constituents: PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA

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.

The GBE1 protein also known as glycogen branching enzyme 1 plays an important role in glycogen metabolism. It has a molecular mass of approximately 83 kDa. The enzyme catalyzes the formation of alpha-16-glycosidic linkages in glycogen adding branches to the linear chains of glucose residues. This branching increases the solubility of glycogen and improves its accessibility for degradation. GBE1 expression occurs mainly in the liver and muscle tissues where glycogen storage and metabolism are vital for energy balance and function.

Biological function summary

The enzyme's activity ensures the efficient storage and mobilization of glycogen serving an important role in energy regulation. It does not operate as part of a larger enzymatic complex but works alone to modify the structure of glycogen. Its activity impacts the balance between corticosteroid glucose production and glycogen storage thereby playing a part in maintaining glucose homeostasis in the body.

Pathways

GBE1 participates in the glycogen biosynthesis pathway which is vital for energy storage in organisms. The protein acts alongside others like glycogen synthase to ensure proper glycogen formation which serves as a significant energy reserve. Also GBE1 relates to the gluconeogenesis/glycogenolysis pathway where its role in structuring glycogen ensures substrates are readily available when needed for energy production during fasting or rigorous activity.

Mutations or malfunctions in the GBE1 gene can lead to Glycogen Storage Disease type IV (Andersen's disease) a disorder affecting glycogen metabolism. Symptoms can include hepatic and muscular dysfunctions due to improperly structured glycogen. The condition is associated with another protein glycogen synthase because both contribute to abnormal glycogen accumulation. Also research links enzyme deficits to adult polyglucosan body disease where abnormal glycogen bodies accumulate in the nervous system leading to neurological issues.

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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

Glycogen-branching enzyme participates in the glycogen biosynthetic process along with glycogenin and glycogen synthase. Generates alpha-1,6-glucosidic branches from alpha-1,4-linked glucose chains, to increase solubility of the glycogen polymer (PubMed : 26199317, PubMed : 8463281, PubMed : 8613547).

See full target information 1,4-alpha-glucan-branching enzyme

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文献 (11)

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

Cancers 15: PubMed36900384

2023

GBE1 Promotes Glioma Progression by Enhancing Aerobic Glycolysis through Inhibition of FBP1.

Applications

Unspecified application

Species

Unspecified reactive species

Zhen Chen,Han Bao,Jingfang Long,Peiqi Zhao,Xiaowei Hu,Hao Wang,Ying Zhang,Jianjing Yang,Qichuan Zhuge,Lei Xia

Disease models & mechanisms 16: PubMed36511140

2023

Laforin targets malin to glycogen in Lafora progressive myoclonus epilepsy.

Applications

Unspecified application

Species

Unspecified reactive species

Sharmistha Mitra,Baozhi Chen,Peixiang Wang,Erin E Chown,Mathew Dear,Dikran R Guisso,Ummay Mariam,Jun Wu,Emrah Gumusgoz,Berge A Minassian

Neuropathology and applied neurobiology 49:e12865 PubMed36456471

2022

Abundant copathologies of polyglucosan bodies, frontotemporal lobar degeneration with TDP-43 inclusions and ageing-related tau astrogliopathy in a family with a GBE1 mutation.

Applications

Unspecified application

Species

Unspecified reactive species

Maiko T Uemura,Eun Ran Suh,John L Robinson,Kurt R Brunden,Murray Grossman,David J Irwin,Virginia M-Y Lee,John Q Trojanowski,Edward B Lee,Vivianna M Van Deerlin

Brain : a journal of neurology 145:2361-2377 PubMed35084461

2022

Glycogen synthase downregulation rescues the amylopectinosis of murine RBCK1 deficiency.

Applications

Unspecified application

Species

Unspecified reactive species

Silvia Nitschke,Mitchell A Sullivan,Sharmistha Mitra,Charlotte R Marchioni,Jennifer P Y Lee,Brandon H Smith,Saija Ahonen,Jun Wu,Erin E Chown,Peixiang Wang,Sara Petković,Xiaochu Zhao,Laura F DiGiovanni,Ami M Perri,Lori Israelian,Tamar R Grossman,Holly Kordasiewicz,Francisco Vilaplana,Kazuhiro Iwai,Felix Nitschke,Berge A Minassian

International journal of molecular sciences 22: PubMed34502525

2021

Mice with Whole-Body Disruption of AMPK-Glycogen Binding Have Increased Adiposity, Reduced Fat Oxidation and Altered Tissue Glycogen Dynamics.

Applications

Unspecified application

Species

Unspecified reactive species

Natalie R Janzen,Jamie Whitfield,Lisa Murray-Segal,Bruce E Kemp,John A Hawley,Nolan J Hoffman

American journal of human genetics 108:1301-1317 PubMed34038740

2021

Loss of C2orf69 defines a fatal autoinflammatory syndrome in humans and zebrafish that evokes a glycogen-storage-associated mitochondriopathy.

Applications

Unspecified application

Species

Unspecified reactive species

Hui Hui Wong,Sze Hwee Seet,Michael Maier,Ayse Gurel,Ricardo Moreno Traspas,Cheryl Lee,Shan Zhang,Beril Talim,Abigail Y T Loh,Crystal Y Chia,Tze Shin Teoh,Danielle Sng,Jarred Rensvold,Sule Unal,Evgenia Shishkova,Ece Cepni,Fatima M Nathan,Fernanda L Sirota,Chao Liang,Nese Yarali,Pelin O Simsek-Kiper,Tadahiro Mitani,Serdar Ceylaner,Ozlem Arman-Bilir,Hamdi Mbarek,Fatma Gumruk,Stephanie Efthymiou,Deniz Uğurlu Çi Men,Danai Georgiadou,Kortessa Sotiropoulou,Henry Houlden,Franziska Paul,Davut Pehlivan,Candice Lainé,Guoliang Chai,Nur Ain Ali,Siew Chin Choo,Soh Sok Keng,Bertrand Boisson,Elanur Yılmaz,Shifeng Xue,Joshua J Coon,Thanh Thao Nguyen Ly,Naser Gilani,Dana Hasbini,Hulya Kayserili,Maha S Zaki,Robert J Isfort,Natalia Ordonez,Kornelia Tripolszki,Peter Bauer,Nima Rezaei,Simin Seyedpour,Ghamar Taj Khotaei,Charles C Bascom,Reza Maroofian,Myriam Chaabouni,Afaf Alsubhi,Wafaa Eyaid,Sedat Işıkay,Joseph G Gleeson,James R Lupski,Jean-Laurent Casanova,David J Pagliarini,Nurten A Akarsu,Sebastian Maurer-Stroh,Arda Cetinkaya,Aida Bertoli-Avella,Ajay S Mathuru,Lena Ho,Frederic A Bard,Bruno Reversade

Neuroscience bulletin 36:1513-1523 PubMed33048310

2020

Dynamic Variations in Brain Glycogen are Involved in Modulating Isoflurane Anesthesia in Mice.

Applications

Unspecified application

Species

Unspecified reactive species

Ze Fan,Zhihao Zhang,Shiyi Zhao,Yuanyuan Zhu,Dong Guo,Bo Yang,Lixia Zhuo,Jiao Han,Rui Wang,Zongping Fang,Hailong Dong,Yan Li,Lize Xiong

Signal transduction and targeted therapy 5:54 PubMed32439898

2020

Hypoxia-induced GBE1 expression promotes tumor progression through metabolic reprogramming in lung adenocarcinoma.

Applications

Unspecified application

Species

Unspecified reactive species

Lifeng Li,Li Yang,Zhirui Fan,Wenhua Xue,Zhibo Shen,Yongliang Yuan,Xiangdong Sun,Dan Wang,Jingyao Lian,Liping Wang,Jie Zhao,Yi Zhang

Cell reports 27:1334-1344.e6 PubMed31042462

2019

Skeletal Muscle Glycogen Chain Length Correlates with Insolubility in Mouse Models of Polyglucosan-Associated Neurodegenerative Diseases.

Applications

Unspecified application

Species

Unspecified reactive species

Mitchell A Sullivan,Silvia Nitschke,Evan P Skwara,Peixiang Wang,Xiaochu Zhao,Xiao S Pan,Erin E Chown,Travis Wang,Ami M Perri,Jennifer P Y Lee,Francisco Vilaplana,Berge A Minassian,Felix Nitschke

American journal of physiology. Regulatory, integr 311:R307-14 PubMed27280431

2016

Differential glucose metabolism in mice and humans affected by McArdle disease.

Applications

Unspecified application

Species

Unspecified reactive species

Thomas O Krag,Tomàs Pinós,Tue L Nielsen,Jordi Duran,Mar García-Rocha,Antoni L Andreu,John Vissing

View all publications

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Anti-GBE1抗体[EP11113]