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Galactosylceramidase Recombinant Rabbit mAb (S-3402-17)

Galactocerebrosidase,GALCERase,Galactocerebroside beta-galactosidase,Galactosylceramide beta-galactosidase,Galc

价格 600.00 供应商现货 : 3-5个工作日
货号 S0B6671
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产品介绍 评论(0)

产品规格
  • 宿主来源

    Rabbit
  • 抗原名称

    Galactosylceramidase
  • 分子别名

    Galactocerebrosidase; GALCERase; Galactocerebroside beta-galactosidase; Galactosylceramide beta-galactosidase; Galc
  • 免疫原

    Recombinant Protein
  • 细胞定位

    Lysosome
  • Accession

    P54818
  • 克隆号

    S-3402-17
  • 抗体类型

    Recombinant mAb
  • 抗体同种型

    IgG
  • 反应种属 ?

    Hu, Ms, Rt, Mk
  • 阳性样本

    A549, SH-SY5Y, A375, HepG2, mouse brain, COS-7
  • 纯化方式

    Protein A
  • 浓度

    0.5 mg/ml
  • 标记

    Unconjugated
  • 性状

    Liquid
  • 缓冲体系

    PBS, 40% Glycerol, 0.05% BSA, 0.03% Proclin 300

  • 储存条件

    12 months from date of receipt / reconstitution, -20 °C as supplied

  • 应用

    IHC-P ?

    WB

  • 稀释度

    应用 稀释度 推荐种属
    WB 1:1000 Hu, Ms, Mk
    IHC-P 1:1000 Hu, Ms, Rt
背景介绍
  • Galactosylceramidase, also known as galactocerebrosidase (GALC), is a lysosomal enzyme encoded by the GALC gene on chromosome 14q31.3 that catalyzes the hydrolytic cleavage of galactose from galactosylceramide and other galactolipids, playing a crucial role in myelin maintenance and turnover; deficiency in this enzyme leads to the accumulation of toxic metabolites, particularly psychosine, resulting in Krabbe disease (globoid cell leukodystrophy), a severe neurodegenerative disorder characterized by demyelination, multinucleated globoid cells in the white matter, and progressive loss of motor and cognitive functions, with early-onset forms typically manifesting within the first six months of life and proving fatal within a few years.

  • 免疫印迹

    • WB result of Galactosylceramidase Recombinant Rabbit mAb
      Primary antibody: Galactosylceramidase Recombinant Rabbit mAb at 1/1000 dilution
      Lane 1: A549 whole cell lysate 20 µg
      Lane 2: SH-SY5Y whole cell lysate 20 µg
      Lane 3: A375 whole cell lysate 20 µg
      Lane 4: HepG2 whole cell lysate 20 µg
      Secondary antibody: Goat Anti- rabbit IgG, (H+L), HRP conjugated at 1/10000 dilution
      Predicted MW: 77 kDa
      Observed MW: 30, 50, 70, 170 kDa

    • WB result of Galactosylceramidase Recombinant Rabbit mAb
      Primary antibody: Galactosylceramidase Recombinant Rabbit mAb at 1/1000 dilution
      Lane 1: mouse brain lysate 20 µg
      Secondary antibody: Goat Anti- rabbit IgG, (H+L), HRP conjugated at 1/10000 dilution
      Predicted MW: 77 kDa
      Observed MW: 30, 50, 65 kDa

    • WB result of Galactosylceramidase Recombinant Rabbit mAb
      Primary antibody: Galactosylceramidase Recombinant Rabbit mAb at 1/1000 dilution
      Lane 1: COS-7 whole cell lysate 20 µg
      Secondary antibody: Goat Anti- rabbit IgG, (H+L), HRP conjugated at 1/10000 dilution
      Predicted MW: 77 kDa
      Observed MW: 30, 50 kDa

  • 免疫组化

    • IHC shows positive staining in paraffin-embedded human cerebral cortex. Anti-Galactosylceramidase antibody was used at 1/1000 dilution, followed by a HRP Polymer for Mouse & Rabbit IgG (ready to use). Counterstained with hematoxylin. Heat mediated antigen retrieval with Tris/EDTA buffer pH9.0 was performed before commencing with IHC staining protocol.

    • IHC shows positive staining in paraffin-embedded human hepatocellular carcinoma. Anti-Galactosylceramidase antibody was used at 1/1000 dilution, followed by a HRP Polymer for Mouse & Rabbit IgG (ready to use). Counterstained with hematoxylin. Heat mediated antigen retrieval with Tris/EDTA buffer pH9.0 was performed before commencing with IHC staining protocol.

    • IHC shows positive staining in paraffin-embedded mouse cerebral cortex. Anti-Galactosylceramidase antibody was used at 1/1000 dilution, followed by a HRP Polymer for Mouse & Rabbit IgG (ready to use). Counterstained with hematoxylin. Heat mediated antigen retrieval with Tris/EDTA buffer pH9.0 was performed before commencing with IHC staining protocol.

    • IHC shows positive staining in paraffin-embedded rat cerebral cortex. Anti-Galactosylceramidase antibody was used at 1/1000 dilution, followed by a HRP Polymer for Mouse & Rabbit IgG (ready to use). Counterstained with hematoxylin. Heat mediated antigen retrieval with Tris/EDTA buffer pH9.0 was performed before commencing with IHC staining protocol.

  • 组织表达图谱

    • Expression of Galactosylceramidase in tumor tissue.

    • Expression of Galactosylceramidase in human tissue.

    • Expression of Galactosylceramidase in mouse & rat tissue.

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