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NDUFV1 Antibody

  • 中文名稱：
    NDUFV1兔多克隆抗體
  • 貨號(hào)：
    CSB-PA015668ESR2HU
  • 規(guī)格：
    ￥440
  • 促銷：
    小規(guī)格抗體限時(shí)一口價(jià)
  • 圖片：
    • Western blot
      All lanes: NDUFV1 antibody at 3.46µg/ml + HepG2 whole cell lysate
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 51, 50 kDa
      Observed band size: 51 kDa
    • Immunohistochemistry of paraffin-embedded human brain tissue using CSB-PA015668ESR2HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA015668ESR2HU at dilution of 1:100
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) NDUFV1 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
  • 別名：
    CI 51kD antibody; CI-51kD antibody; CI51KD antibody; Complex I 51kD antibody; Complex I-51kD antibody; FLJ59059 antibody; mitochondrial antibody; NADH dehydrogenase (ubiquinone) flavoprotein 1 antibody; NADH dehydrogenase [ubiquinone] flavoprotein 1 antibody; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial antibody; NADH dehydrogenase flavoprotein 1 antibody; NADH ubiquinone oxidoreductase 51 kDa subunit antibody; NADH ubiquinone oxidoreductase antibody; NADH ubiquinone oxidoreductase core subunit V1 antibody; NADH-ubiquinone oxidoreductase 51 kDa subunit antibody; NDUFV 1 antibody; ndufv1 antibody; NDUV1_HUMAN antibody; UQOR1 antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial protein (1-250AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    Non-conjugated
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    Antigen Affinity Purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA, WB, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:1000-1:5000
    IHC 1:20-1:200
  • Protocols：
  • 儲(chǔ)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.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

  • 功能：
    Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor.
  • 基因功能參考文獻(xiàn)：
    1. Mutations in the ND6, NDUFV1 or ACAD9 genes are responsible for the mitochondrial complex I deficiency. PMID: 29348607
    2. we have used a yeast model system to study the molecular consequences of 16 single amino acid substitutions, classified as pathogenic, in the NDUFV1 subunit of complex I PMID: 26345448
    3. small number of putative de novo variants were transmitted from BAP parents to their ASD offspring, and evidence emerged for a rare duplication CNV at 11p13.3 harboring two putative developmental/neuropsychiatric susceptibility gene(s), GSTP1 and NDUFV1. PMID: 25432440
    4. The results affirm that NDUFV1 mutations are causative of the phenotype in two siblings affected by a diffuse leukodystrophy. PMID: 23562761
    5. observed 2 consanguinous siblings with early-onset encephalopathy, medulla, brainstem and mesencephalon lesions and death before 8 months of age, caused by a complex I deficiency; identified a missense mutation in the NDUFV1 gene; the mutation, p.Arg386His, affects a highly conserved residue PMID: 21696386
    6. study describes clinical, radiological, biochemical and molecular data of 6 patients with Leigh syndrome with novel mutations in NDUFV1 and NDUFS2; 2 siblings were homozygous for previously undescribed R386C mutation in NDUFV1 PMID: 23266820
    7. significant negative-correlation between left ventricular end-diastolic dimension and NDUFV1 production in dilated cardiomyopathy PMID: 20930427
    8. Sp1 was abnormally expressed in schizophrenia and its mRNA alteration pattern paralleled that of NDUFV1 and NDUFV2 in schizophrenic patients. PMID: 17786189
    9. Mutations in the NDUFV1 gene is linked to a delayed mitochondrial network recovery in OXPHOS disorders. PMID: 20153825

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  • 相關(guān)疾病：
    Leigh syndrome (LS); Mitochondrial complex I deficiency (MT-C1D)
  • 亞細(xì)胞定位：
    Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
  • 蛋白家族：
    Complex I 51 kDa subunit family
  • 數(shù)據(jù)庫(kù)鏈接：

    HGNC: 7716

    OMIM: 161015

    KEGG: hsa:4723

    STRING: 9606.ENSP00000322450

    UniGene: Hs.7744