Recombinant人NDUFV1 protein (ab117059)

概述

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP49821
    • 种属Human
    • 序列KAIARLIEFYKHESCGQCTPCREGVDWMNKVMARFVRGDARPAEIDSLWE ISKQIEGHTICALGDGAAWPVQGLIRHFRPELEERMQRFAQQHQARQAAS
    • 分子量37 kDa including tags
    • 氨基酸365 to 464

相关产品

技术指标

Our Abpromise guarantee covers the use of ab117059 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    Western blot

    SDS-PAGE

    ELISA

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • CI 51kD
    • CI-51kD
    • Complex I 51kD
    • Complex I-51kD
    • FLJ59059
    • mitochondrial
    • NADH dehydrogenase (ubiquinone) flavoprotein 1
    • NADH dehydrogenase [ubiquinone] flavoprotein 1
    • NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial
    • NADH dehydrogenase flavoprotein 1
    • NADH ubiquinone oxidoreductase
    • NADH ubiquinone oxidoreductase 51 kDa subunit
    • NADH-ubiquinone oxidoreductase 51 kDa subunit
    • NDUFV 1
    • ndufv1
    • NDUV1_HUMAN
    • UQOR1
    see all
  • 功能Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for 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.
  • 疾病相关Defects in NDUFV1 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.
    Defects in NDUFV1 are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease.
  • 序列相似性Belongs to the complex I 51 kDa subunit family.
  • 细胞定位Mitochondrion inner membrane.
  • Information by UniProt

Recombinant Human NDUFV1 protein 图像

  • 12.5% SDS-PAGE showing ab117059 at approximately 36.63 kDa.
    Stained with Coomassie Blue.

Recombinant Human NDUFV1 protein (ab117059)参考文献

ab117059 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab117059.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"