NDUFA1 blocking peptide

NDUFA1 Blocking Peptide (Center)

• Gene Names: NDUFA1; MWFE; ZNF183; CI-MWFE
• Synonyms: NDUFA1; NDUFA1 Blocking Peptide (Center); NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1; Complex I-MWFE; CI-MWFE; NADH-ubiquinone oxidoreductase MWFE subunit; NDUFA1 blocking peptide

• Specificity: The synthetic peptide sequence is selected from aa 46-59 of HUMAN NDUFA1
• 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: 70

• Cellular Location: Mitochondrion inner membrane; Single-pass membrane protein; Matrix side
• Tissue Location: Primarily expressed in heart and skeletal muscle
• 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 NDUFA1 blocking peptide

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.

NCBI and Uniprot Product Information

• NCBI GI #: 3334271
• NCBI GeneID: 4694
• UniProt Accession #: O15239
• Molecular Weight: 8,072 Da
• NCBI Official Full Name: NADH dehydrogenase
• NCBI Official Synonym Full Names: NADH:ubiquinone oxidoreductase subunit A1
• NCBI Official Symbol: NDUFA1
• NCBI Official Synonym Symbols: MWFE; ZNF183; CI-MWFE
• NCBI Protein Information: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1
• UniProt Protein Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1
• UniProt Gene Name: NDUFA1
• UniProt Synonym Gene Names: CI-MWFE
• UniProt Entry Name: NDUA1_HUMAN

NCBI Description

The human NDUFA1 gene codes for an essential component of complex I of the respiratory chain, which transfers electrons from NADH to ubiquinone. It has been noted that the N-terminal hydrophobic domain has the potential to be folded into an alpha-helix spanning the inner mitochondrial membrane with a C-terminal hydrophilic domain interacting with globular subunits of complex I. The highly conserved two-domain structure suggests that this feature is critical for the protein function and might act as an anchor for the NADH:ubiquinone oxidoreductase complex at the inner mitochondrial membrane. However, the NDUFA1 peptide is one of about 31 components of the "hydrophobic protein" (HP) fraction of complex I which is involved in proton translocation. Thus the NDUFA1 peptide may also participate in that function. [provided by RefSeq, Jul 2008]

Uniprot Description

NDUFA1: 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 NDUFA1 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 NDUFA1 subunit family.

Protein type: Mitochondrial; EC 1.6.5.3; Membrane protein, integral; Oxidoreductase; Energy Metabolism - oxidative phosphorylation; EC 1.6.99.3

Chromosomal Location of Human Ortholog: Xq24

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

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

Disease: Mitochondrial Complex I Deficiency