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  4. NDUFB11 Antibody, FITC conjugated

NDUFB11 Antibody, FITC conjugated

  • 中文名稱：
    NDUFB11兔多克隆抗體, FITC偶聯
  • 貨號：
    CSB-PA868343LC01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) NDUFB11 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
  • 別名：
    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
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial protein (30-88AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    FITC
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 產品提供形式：
    Liquid
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    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.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 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.
  • 基因功能參考文獻：
    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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  • 相關疾病：
    Linear skin defects with multiple congenital anomalies 3 (LSDMCA3); Mitochondrial complex I deficiency (MT-C1D)
  • 亞細胞定位：
    Mitochondrion inner membrane; Single-pass membrane protein.
  • 蛋白家族：
    Complex I NDUFB11 subunit family
  • 組織特異性：
    Ubiquitous.
  • 數據庫鏈接：

    HGNC: 20372

    OMIM: 252010

    KEGG: hsa:54539

    UniGene: Hs.521969



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