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GBA Antibody (monoclonal) (M01

)
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  • $495
  • Leading Biology
  • United States
  • AMM04718G
  • 2026年03月31日
  • Mouse
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    • 详细信息
    • 文献和实验
    • 技术资料
    • 亚型

      IgG2a Kappa

    • 保存条件

      Store at +4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

    • 克隆性

      单克隆

    • 库存

      100

    • 宿主

      Mouse

    • 靶点

      GBA (NP_000148, 146 a.a. ~ 235 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

    • 规格

      100 μg

    分子量:
    59716 Da
    细胞定位:

    形式:
    Clear, colorless solution in phosphate buffered saline, pH 7.2 .
    参考文献:
    1.Parkin-mediated ubiquitination of mutant glucocerebrosidase leads to competition with its substrates PARIS and ARTS.Bendikov-Bar I, Rapaport D, Larisch S, Horowitz MOrphanet J Rare Dis. 2014 Jun 16;9:86. doi: 10.1186/1750-1172-9-86.2.Reduced glucocerebrosidase is associated with increased α-synuclein in sporadic Parkinson's disease.Murphy KE, Gysbers AM, Abbott SK, Tayebi N, Kim WS, Sidransky E, Cooper A, Garner B, Halliday GMBrain. 2014 Jan 28.3.Gaucher disease paradigm: From ERAD to comorbidity.Bendikov-Bar I, Horowitz M.Hum Mutat. 2012 Oct;33(10):1398-407. doi: 10.1002/humu.22124. Epub 2012 Jun 11.4.Characterization of the ERAD process of the L444P mutant glucocerebrosidase variant.Bendikov-Bar I, Ron I, Filocamo M, Horowitz M.Blood Cells Mol Dis. 2010 Nov 22. [Epub ahead of print]5.Characterization of Gaucher disease bone marrow mesenchymal stromal cells reveals an altered inflammatory secretome.Campeau PM, Rafei M, Boivin MN, Sun Y, Grabowski GA, Galipeau J.Blood. 2009 Oct 8;114(15):3181-90. Epub 2009 Jul 8.
    产品概述:
    We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format. We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team. This product is a high quality GBA antibody (monoclonal) (M01).
    功能:

    总结:
    This gene encodes a lysosomal membrane protein that cleaves the beta-glucosidic linkage of glycosylceramide, an intermediate in glycolipid metabolism. Mutations in this gene cause Gaucher disease, a lysosomal storage disease characterized by an accumulation of glucocerebrosides. A related pseudogene is approximately 12 kb downstream of this gene on chromosome 1. Alternative splicing results in multiple transcript variants.
    联系方式:

    生物商城:http://www.annoron.com

    办公电话:010-56256916

    官方售后:400-965-8633

    企业 QQ:3212874516、3198592576

    地 址:北京经济技术开发区永昌中路4号院4号楼3层

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    图标文献和实验
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