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NDUFA1 Antibody

  • 货号：
    CSB-PA271484
  • 规格：
    ￥1100
  • 图片：
    • The image on the left is immunohistochemistry of paraffin-embedded Human colon cancer tissue using CSB-PA271484(NDUFA1 Antibody) at dilution 1/30, on the right is treated with fusion protein. (Original magnification: ×200)
    • The image on the left is immunohistochemistry of paraffin-embedded Human brain tissue using CSB-PA271484(NDUFA1 Antibody) at dilution 1/30, on the right is treated with fusion protein. (Original magnification: ×200)
  • 其他：

产品详情

  • Uniprot No.：
    O15239
  • 基因名：
  • 别名：
    NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1; Complex I-MWFE; CI-MWFE; NADH-ubiquinone oxidoreductase MWFE subunit
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Fusion protein of Human NDUFA1
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen affinity purification
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,IHC
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:2000-1:5000
    IHC 1:50-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
  • 基因功能参考文献：
    1. Results from a study on gene expression variability markers in early-stage human embryos shows that NDUF1A is a putative expression variability marker for the 3-day, 8-cell embryo stage. PMID: 26288249
    2. The gene signature of OPA1, CTSA, NDUFA1, STK10 and PRDX1 was able to identify patients post-implant with a sensitivity of 91% and a specificity of 86% in discrimination between post-implant group and healthy controls. PMID: 27177495
    3. Fanconi anemia complementation group A mutants show defective respiration through Complex I, diminished ATP production and metabolic sufferance with an increased AMP/ATP ratio. PMID: 23791750
    4. Heterozygous mutation in the X chromosomal NDUFA1 gene in a girl with complex I deficiency PMID: 21596602
    5. Mutations in the NDUFA1 gene is linked to a delayed mitochondrial network recovery in OXPHOS disorders. PMID: 20153825
    6. Species-specific and mutant MWFE proteins effect on the assembly of a functional mammalian mitochondrial complex I. PMID: 11937507
    7. A family-based association study finds that the NDUFA1 gene is unlikely to harbor a major visual loss susceptibility locus for Leber hereditary optic neuropathy. PMID: 12084895
    8. Oxidative stress and partial deficiencies of mitochondrial complex I are key factors in the pathogenesis of Parkinson's disease. (REVIEW) PMID: 15038604
    9. Suppression of NDUFA1 expression could represent a key pathogenic mechanism in the development of basal cell carcinoma PMID: 15854127
    10. Two novel p.Gly8Arg and p.Arg37Ser hemizygous mutations in NDUFA1 were identified in two unrelated male patients presenting with Leigh's syndrome and with myoclonic epilepsy and developmental delay. PMID: 17262856
    11. hypothesize that the novel G32R mutation in NDUFA1 is causing complex I deficiency either by itself or in synergy with additional mtDNA variants PMID: 19185523

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  • 相关疾病：
    Mitochondrial complex I deficiency (MT-C1D)
  • 亚细胞定位：
    Mitochondrion inner membrane; Single-pass membrane protein; Matrix side.
  • 蛋白家族：
    Complex I NDUFA1 subunit family
  • 组织特异性：
    Primarily expressed in heart and skeletal muscle.
  • 数据库链接：

    HGNC: 7683

    OMIM: 252010

    KEGG: hsa:4694

    STRING: 9606.ENSP00000360492

    UniGene: Hs.534168