NDUFV1 blocking peptide

NDUFV1 Blocking Peptide (N-term)

• Gene Names: NDUFV1; UQOR1; CI-51K; CI51KD
• Synonyms: NDUFV1; NDUFV1 Blocking Peptide (N-term); NADH dehydrogenase [ubiquinone] flavoprotein 1; mitochondrial; Complex I-51kD; CI-51kD; NADH dehydrogenase flavoprotein 1; NADH-ubiquinone oxidoreductase 51 kDa subunit; UQOR1; NDUFV1 blocking peptide

• Specificity: The synthetic peptide sequence is selected from aa 22-36 of HUMAN NDUFV1
• Form/Format: Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
• Sequence Length: 464

• Cellular Location: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for NDUFV1 blocking peptide

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).

NCBI and Uniprot Product Information

• NCBI GI #: 20455501
• NCBI GeneID: 4723
• UniProt Accession #: P49821; O60924; O60940; Q16104; Q6IBR3; Q96BF8; Q96HS7
• Molecular Weight: 49,868 Da
• NCBI Official Full Name: NADH dehydrogenase
• NCBI Official Synonym Full Names: NADH:ubiquinone oxidoreductase core subunit V1
• NCBI Official Symbol: NDUFV1
• NCBI Official Synonym Symbols: UQOR1; CI-51K; CI51KD
• NCBI Protein Information: NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial
• UniProt Protein Name: NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial
• UniProt Gene Name: NDUFV1
• UniProt Synonym Gene Names: UQOR1; CI-51kD
• UniProt Entry Name: NDUV1_HUMAN

NCBI Description

The mitochondrial respiratory chain provides energy to cells via oxidative phosphorylation and consists of four membrane-bound electron-transporting protein complexes (I-IV) and an ATP synthase (complex V). This gene encodes a 51 kDa subunit of the NADH:ubiquinone oxidoreductase complex I; a large complex with at least 45 nuclear and mitochondrial encoded subunits that liberates electrons from NADH and channels them to ubiquinone. This subunit carries the NADH-binding site as well as flavin mononucleotide (FMN)- and Fe-S-biding sites. Defects in complex I are a common cause of mitochondrial dysfunction; a syndrome that occurs in approximately 1 in 10,000 live births. Mitochondrial complex I deficiency is linked to myopathies, encephalomyopathies, and neurodegenerative disorders such as Parkinson's disease and Leigh syndrome. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Oct 2009]

Uniprot Description

NDUFV1: 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. Defects in NDUFV1 are a cause of Leigh syndrome (LS). 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). 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 51 kDa subunit family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial; EC 1.6.5.3; Oxidoreductase; Energy Metabolism - oxidative phosphorylation; EC 1.6.99.3

Chromosomal Location of Human Ortholog: 11q13

Cellular Component: mitochondrial inner membrane; mitochondrial respiratory chain complex I

Molecular Function: 4 iron, 4 sulfur cluster binding; FMN binding; NAD binding; NADH dehydrogenase activity

Biological Process: aerobic respiration; mitochondrial electron transport, NADH to ubiquinone; mitochondrial respiratory chain complex I assembly

Disease: Mitochondrial Complex I Deficiency