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  4. Recombinant Human NADH-ubiquinone oxidoreductase chain 3 (MT-ND3)

Recombinant Human NADH-ubiquinone oxidoreductase chain 3 (MT-ND3)

  • 中文名称：
    人MT-ND3重组蛋白
  • 货号：
    CSB-CF015078HU
  • 规格：
  • 来源：
    in vitro E.coli expression system
  • 其他：

产品详情

  • 基因名：
  • Uniprot No.：
  • 别名：
    MT-ND3; MTND3; NADH3; ND3; NADH-ubiquinone oxidoreductase chain 3; NADH dehydrogenase subunit 3
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    full length protein
  • 表达区域：
    1-115
  • 氨基酸序列
    MNFALILMINTLLALLLMIITFWLPQLNGYMEKSTPYECGFDPMSPARVPFSMKFFLVAI TFLLFDLEIALLLPLPWALQTTNLPLMVMSSLLLIIILALSLAYEWLQKGLDWTE
    Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
  • 蛋白标签：
    N-terminal 10xHis-tagged
  • 产品提供形式：
    Liquid or Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 缓冲液：
    Lyophilized from Tris/PBS-based buffer, 6% Trehalose, pH 8.0
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet & COA：
    Please contact us to get it.

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. Essential for the catalytic activity of complex I.
  • 基因功能参考文献：
    1. Disruption of FASTKD1 increases ND3 mRNA level. Disruption of FASTKD4 reduces it. Very low levels of FASTKD4 are sufficient to prevent ND3 loss. PMID: 28335001
    2. In the current study, we first took clinical and molecular datasets from case-control studies to determine the association between the ND3 G10398A mutation and breast cancer. PMID: 26782384
    3. Earlier age at onset was noted in male Chinese Machado-Joseph disease patients with MT-ND3 gene 10398A polymorphism PMID: 26336829
    4. The 10398G allele and Haplogroup I appear to confer significant protective effects for Fuchs endothelial corneal dystrophy PMID: 24917144
    5. Hypoxia, oxidative stress, and saturated fatty acids impacted mitochondrial-mediated cell apoptosis and had promotion on MT-ND3 expression in hepatocytes. PMID: 24020820
    6. Point mutations m.10191T>C in mitochondrial ND3 gene, m.13513G>A in ND5 gene and m.14,453G>A in ND6 gene were detected in three Chinese children with Leigh synrome dur to complex I deficiency. PMID: 22947169
    7. this short clinical review we evaluate the case reports of the m.10191T>C mutation causing complex I-deficient Leigh syndrome described in the literature. PMID: 22364517
    8. mitochondrial ND3 gene mutation may have a role in causing in Leigh syndrome with early lethality PMID: 14764913
    9. This is the first description of infantile Leigh syndrome due to a maternally transmitted T10191C substitution in ND3 and not due to a de novo mutation. PMID: 16023078
    10. These results show that the 10197G>A mutation in the mitochondrial ND3 gene should be considered as a common mtDNA mutation responsible for Leigh syndrome and dystonia. PMID: 17152068
    11. study reports a novel heteroplasmic m.10197G>A mutation in the ND3 gene in three Korean children with bilateral basal ganglia lesions and partial deficiencies of respiratory chain complex I activity PMID: 17413873
    12. These findings suggest that the clinical presentations associated with the mtND3*10197A (m.10197G>A) mutation (ND3) are much wider, encompassing those of LDYT and Leigh syndrome. PMID: 19458970
    13. 2 cases with a MELAS-like phenotype with additional unique features of epilepsia partialis continua accompanied by evolving lesions of the rolandic & calcarine cortices; both carried mutations in the MT-ND3 gene -- mt.10158T>C & mt.10191T>C PMID: 19520270
    14. mutations in the ND3 and ND5 genes in patients showing clinical features of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes PMID: 19617458

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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; Multi-pass membrane protein.
  • 蛋白家族：
    Complex I subunit 3 family
  • 数据库链接：

    HGNC: 7458

    OMIM: 252010

    KEGG: hsa:4537

    STRING: 9606.ENSP00000355206