Human NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase) ELISA Kit | NDUFS1 elisa kit
Human NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial, NDUFS1 ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene 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. This protein 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 this gene are associated with complex I deficiency. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jan 2011]
Uniprot Description
NDUFS1: 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. 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. Defects in NDUFS1 are a cause of mitochondrial complex I deficiency (MT-C1D). 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. Belongs to the complex I 75 kDa subunit family. 4 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 1.6.99.3; Mitochondrial; EC 1.6.5.3; Oxidoreductase; Energy Metabolism - oxidative phosphorylation
Chromosomal Location of Human Ortholog: 2q33-q34
Cellular Component: mitochondrion; mitochondrial inner membrane; mitochondrial intermembrane space; mitochondrial respiratory chain complex I
Molecular Function: 2 iron, 2 sulfur cluster binding; protein binding; NADH dehydrogenase (ubiquinone) activity; electron carrier activity; 4 iron, 4 sulfur cluster binding; metal ion binding
Biological Process: ATP metabolic process; cellular metabolic process; apoptotic mitochondrial changes; regulation of mitochondrial membrane potential; mitochondrial electron transport, NADH to ubiquinone; cellular respiration
Disease: Mitochondrial Complex I Deficiency
Research Articles on NDUFS1
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Product Notes
The Human NDUFS1 ndufs1 (Catalog #AAA9316968) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9316968 ELISA Kit recognizes Human NDUFS1. It is sometimes possible for the material contained within the vial of "NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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