NDUFS4 blocking peptide
NDUFS4 Antibody (C-term) Blocking Peptide
NCBI and Uniprot Product Information
NCBI Description
This gene encodes an nuclear-encoded accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I, or NADH:ubiquinone oxidoreductase). Complex I removes electrons from NADH and passes them to the electron acceptor ubiquinone. Mutations in this gene can cause mitochondrial complex I deficiencies such as Leigh syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2015]
Uniprot Description
NDUFS4: Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in 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. Defects in NDUFS4 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 NDUFS4 subunit family.
Protein type: EC 1.6.99.3; Energy Metabolism - oxidative phosphorylation; Oxidoreductase; Mitochondrial; EC 1.6.5.3
Chromosomal Location of Human Ortholog: 5q11.1
Cellular Component: mitochondrial inner membrane; mitochondrial respiratory chain complex I; mitochondrion
Molecular Function: NADH dehydrogenase (ubiquinone) activity
Biological Process: brain development; cAMP-mediated signaling; cellular respiration; mitochondrial electron transport, NADH to ubiquinone; mitochondrial respiratory chain complex I assembly; positive regulation of fibroblast proliferation; regulation of protein amino acid phosphorylation; response to cAMP
Disease: Leigh Syndrome; Mitochondrial Complex I Deficiency
Research Articles on NDUFS4
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Product Notes
The NDUFS4 ndufs4 (Catalog #AAA9226438) is a Blocking Peptide and is intended for research purposes only. The product is available for immediate purchase. It is sometimes possible for the material contained within the vial of "NDUFS4, Blocking Peptide" 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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