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Ndufs1 Antibody Blocking Pepti

de(bs-3890P)-500ug
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  • 2025年10月16日
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      500ug

    产品编号bs-3890P
    英文名称Ndufs1 Antibody Blocking Peptide
    中文名称线粒体复合物NDUFS1蛋白封闭多肽
    英文别名Complex I 75Kd; Complex I, mitochondrial respiratory chain, 75 kD subunit; NADH coenzyme Q reductase; NADH dehydrogenase (ubiquinone) FeS protein 1 (75kD) (NADH coenzyme Q reductase) ; NADH ubiquinone oxidoreductase 75 kDa subunit mitochondrial; PRO1304.
    纯化方法HPLC
    研究领域

    Cancer > Cancer Metabolism > Cellular metabolic process

    Cancer > Cancer Metabolism > Metabolic signaling pathway > Integration of energy metabolism

    Metabolism > Pathways and Processes > Metabolic signaling pathways > Energy transfer pathways > Integration of energy

    Metabolism > Pathways and Processes > Mitochondrial Metabolism > Mitochondrial markers

    Metabolism > Pathways and Processes > Mitochondrial Metabolism > Oxidative phosphorylation > Complex I

    Neuroscience > Neurology process > Metabolism

    Tags & Cell Markers > Subcellular Markers > Organelles > Mitochondria

    亚细胞定位Mitochondrion inner membrane.
    相似性Defects in NDUFS1 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.
    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 (By similarity). This is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized.
    保存条件Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事项This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    背景资料Ndufs1 belongs to the complex I 75 kDa subunit family. Mammalian complex I is composed of 45 different subunits. It locates at the mitochondrial inner membrane. Ndufs1 has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. This protein is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized. Mutations in Ndufs1 gene are associated with complex I deficiency.

     

     

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