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NDUFA9 Recombinant Mouse mAb (S-5382)

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9,mitochondrial,Complex I-39kD (CI-39kD),NADH-ubiquinone oxidoreductase 39 kDa subunit,NDUFS2L

价格 600.00 1-2周
货号 S0B60187
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产品规格
  • 宿主来源

    Mouse
  • 抗原名称

    NDUFA9
  • 分子别名

    NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial; Complex I-39kD (CI-39kD); NADH-ubiquinone oxidoreductase 39 kDa subunit; NDUFS2L
  • 细胞定位

    Mitochondrion
  • Accession

    Q16795
  • 克隆号

    S-5382
  • 抗体类型

    Recombinant mAb
  • 抗体同种型

    IgG1,k
  • 反应种属 ?

    Hu, Ms, Rt
  • 阳性样本

    WI-38, mouse heart, rat heart
  • 预测反应种属
    (反应种属缩写表)

    Bv
  • 纯化方式

    Protein G
  • 浓度

    1.5 mg/ml
  • 标记

    Unconjugated
  • 性状

    Liquid
  • 缓冲体系

    PBS, 40% Glycerol, 0.05% BSA, 0.02% sodium azide

  • 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 1:1000-1:10000 Hu, Ms, Rt
    IHC-P 1:500 Hu, Ms, Rt
背景介绍
  • NDUFA9 is a mitochondrial protein encoded by the NDUFA9 gene, functioning as a 39-kDa non-catalytic accessory subunit of respiratory chain complex I. After being synthesized in the cytoplasm, this protein is transported to the mitochondrial matrix and is primarily localized to the Q module of complex I, where it plays a critical role in maintaining the structural stability and normal function of complex I. Although NDUFA9 is not directly involved in the catalytic electron transfer reactions, it is essential for the proper assembly of complex I and the stabilization of late assembly intermediates, and it may also participate in recruiting the substrate ubiquinone-10. At the pathological level, homozygous or compound heterozygous mutations in the NDUFA9 gene can lead to nuclear type 26 mitochondrial complex I deficiency (MC1DN26), a disorder inherited in an autosomal recessive manner with a broad clinical spectrum, ranging from neonatal-lethal Leigh syndrome to childhood-onset progressive neurodevelopmental disorders with prominent dystonia. Affected patients typically present with developmental delay, dystonia, seizures, and abnormal MRI signals in the basal ganglia, and the underlying pathogenesis lies in the failure of complex I assembly and a significant reduction in its activity caused by these mutations.

  • 免疫印迹

    • WB result of NDUFA9 Recombinant mouse mAb
      Primary antibody: NDUFA9 Recombinant mouse mAb at 1/1000 dilution
      Lane 1: WI-38 whole cell lysate 20 µg
      Secondary antibody: Goat Anti-mouse IgG, (H+L), HRP conjugated at 1/10000 dilution
      Predicted MW: 43 kDa
      Observed MW: 35 kDa

    • WB result of NDUFA9 Recombinant mouse mAb
      Primary antibody: NDUFA9 Recombinant mouse mAb at 1/1000 dilution
      Lane 1: mouse heart lysate 20 µg
      Secondary antibody: Goat Anti-mouse IgG, (H+L), HRP conjugated at 1/10000 dilution
      Predicted MW: 43 kDa
      Observed MW: 35 kDa

    • WB result of NDUFA9 Recombinant mouse mAb
      Primary antibody: NDUFA9 Recombinant mouse mAb at 1/1000 dilution
      Lane 1: rat heart lysate 20 µg
      Secondary antibody: Goat Anti-mouse IgG, (H+L), HRP conjugated at 1/10000 dilution
      Predicted MW: 43 kDa
      Observed MW: 35 kDa

  • 免疫组化

    • IHC shows positive staining in paraffin-embedded human cardiac muscle. Anti-NDUFA9 antibody was used at 1/500 dilution, followed by a HRP Polymer for Mouse 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 kidney. Anti-NDUFA9 antibody was used at 1/500 dilution, followed by a HRP Polymer for Mouse 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 kidney. Anti-NDUFA9 antibody was used at 1/500 dilution, followed by a HRP Polymer for Mouse 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 skeletal muscle. Anti-NDUFA9 antibody was used at 1/500 dilution, followed by a HRP Polymer for Mouse 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 NDUFA9 in human tissues.

    • Expression of NDUFA9 in mouse & rat tissues.

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