NDUFA12 blocking peptide

NDUFA12 Blocking Peptide (N-term)

• Gene Names: NDUFA12; B17.2; DAP13
• Synonyms: NDUFA12; NDUFA12 Blocking Peptide (N-term); NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12; 13 kDa differentiation-associated protein; Complex I-B172; CI-B172; CIB172; NADH-ubiquinone oxidoreductase subunit B172; DAP13; NDUFA12 blocking peptide

• Specificity: The synthetic peptide sequence is selected from aa 34-48 of HUMAN NDUFA12
• 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: 145

• 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 NDUFA12 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 #: 12229870
• NCBI GeneID: 55967
• UniProt Accession #: Q9UI09; Q53XX0; Q9BRV6; F8VQS7
• Molecular Weight: 7,173 Da
• NCBI Official Full Name: NADH dehydrogenase
• NCBI Official Synonym Full Names: NADH:ubiquinone oxidoreductase subunit A12
• NCBI Official Symbol: NDUFA12
• NCBI Official Synonym Symbols: B17.2; DAP13
• NCBI Protein Information: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12
• UniProt Protein Name: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12
• Protein Family: NADH dehydrogenase
• UniProt Gene Name: NDUFA12
• UniProt Synonym Gene Names: DAP13; CI-B17.2; CIB17.2
• UniProt Entry Name: NDUAC_HUMAN

NCBI Description

This gene encodes a protein which is part of mitochondrial complex 1, part of the oxidative phosphorylation system in mitochondria. Complex 1 transfers electrons to ubiquinone from NADH which establishes a proton gradient for the generation of ATP. Mutations in this gene are associated with Leigh syndrome due to mitochondrial complex 1 deficiency. Pseudogenes of this gene are located on chromosomes 5 and 13. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Apr 2012]

Uniprot Description

NDUFA12: 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 NDUFA12 are the cause of Leigh syndrome (LS). An early-onset progressive neurodegenerative disorder characterized by the presence of focal, bilateral lesions in one or more areas of the central nervous system including the brainstem, thalamus, basal ganglia, cerebellum and spinal cord. Clinical features depend on which areas of the central nervous system are involved and include subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, and dysphagia. Belongs to the complex I NDUFA12 subunit family.

Protein type: EC 1.6.5.3; EC 1.6.99.3; Oxidoreductase; Mitochondrial

Chromosomal Location of Human Ortholog: 12q22

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

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

Disease: Leigh Syndrome