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NDUFB11 Antibody

  • 货号：
    CSB-PA015643GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    Q9NX14
  • 基因名：
  • 别名：
    NDUFB11; UNQ111/PRO1064; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial; Complex I-ESSS; CI-ESSS; NADH-ubiquinone oxidoreductase ESSS subunit; Neuronal protein 17.3; Np17.3; p17.3
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Human NDUFB11
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • 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. recurring mutation, c.276_278del, p.F93del, in NDUFB11, a mitochondrial respiratory complex I-associated protein encoded on the X chromosome, in 5 males with a variably syndromic, normocytic congenital sideroblastic anemia. PMID: 27488349
    2. This is the third report that describes a mutation in NDUFB11, but all are associated with a different phenotype. Our results further expand the molecular spectrum and associated clinical phenotype of NDUFB11 defects. PMID: 27102574
    3. The novel NDUFB11 mutation may cause a complex 1 deficiency in synergy with additional unknown mtDNA variants. PMID: 25921236
    4. Mutations in NDUFB11, encoding a complex I component of the mitochondrial respiratory chain, cause microphthalmia with linear skin defects syndrome. PMID: 25772934
    5. the post-transcriptional regulation of the Ndufb11 gene can be involved in the programmed cell death process PMID: 23246602
    6. NDUFB11 did not seem to influence risk and age at onset of visual loss in a total of 65 individuals from 35 Italian Leber hereditary optic neuropathy patients. PMID: 17292333

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  • 相关疾病：
    Linear skin defects with multiple congenital anomalies 3 (LSDMCA3); Mitochondrial complex I deficiency (MT-C1D)
  • 亚细胞定位：
    Mitochondrion inner membrane; Single-pass membrane protein.
  • 蛋白家族：
    Complex I NDUFB11 subunit family
  • 组织特异性：
    Ubiquitous.
  • 数据库链接：

    HGNC: 20372

    OMIM: 252010

    KEGG: hsa:54539

    UniGene: Hs.521969