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NDUFV1 Antibody

  • 货号：
    CSB-PA914526
  • 规格：
    ￥1100
  • 图片：
    • Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane 1-2: Mouse heart tissue, Mouse brain tissue, Primary antibody: CSB-PA914526(NDUFV1 Antibody) at dilution 1/500, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 30 seconds
  • 其他：

产品详情

  • Uniprot No.：
    P49821
  • 基因名：
  • 别名：
    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
  • 反应种属：
    Human,Mouse
  • 免疫原：
    Fusion protein of Human NDUFV1
  • 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,WB
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:2000-1:5000
    WB 1:500-1:2000
  • 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：
    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.
  • 基因功能参考文献：
    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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  • 相关疾病：
    Leigh syndrome (LS); Mitochondrial complex I deficiency (MT-C1D)
  • 亚细胞定位：
    Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
  • 蛋白家族：
    Complex I 51 kDa subunit family
  • 数据库链接：

    HGNC: 7716

    OMIM: 161015

    KEGG: hsa:4723

    STRING: 9606.ENSP00000322450

    UniGene: Hs.7744