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The human NDUFA5 gene codes for the B13 subunit of complex I of the respiratory chain, which transfers electrons from NADH to ubiquinone.
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NDUFA3 (show NDUFA3 Antibodies), NDUFA5 and NDUFA12 (show NDUFA12 Antibodies) supernumerary subunits are necessary for complex I activity and biogenesis.
At the age of 11 months, mice with a central nervous system knockout of Ndufa5 (Ndufa5 CNS-KO) showed lethargy and loss of motor skills. In these mice cortices, the levels of NDUFA5 protein were reduced to 25% of controls. Fully assembled complex I (CI) levels were also greatly reduced in cortex and CI activity in homogenates was reduced to 60% of controls.
The real-time RT-PCR analysis revealed that the expressions of the NDUFA5 mRNA, but not the ZNF9 (show CNBP Antibodies) mRNA level, were significantly up-regulated in all the tested cell lines derived from HPV-positive cervical cancer, HeLa, SW576, and CaSKi.
The human NDUFA5 gene codes for the B13 subunit of complex I of the respiratory chain, which transfers electrons from NADH to ubiquinone. The high degree of conservation of NDUFA5 extending to plants and fungi indicates its functional significance in the enzyme complex. The protein localizes to the inner mitochondrial membrane as part of the 7 component-containing, water soluble 'iron-sulfur protein' (IP) fraction of complex I, although its specific role is unknown. It is assumed to undergo post-translational removal of the initiator methionine and N-acetylation of the next amino acid. The predicted secondary structure is primarily alpha helix, but the carboxy-terminal half of the protein has high potential to adopt a coiled-coil form. The amino-terminal part contains a putative beta sheet rich in hydrophobic amino acids that may serve as mitochondrial import signal. Related pseudogenes have also been identified on four other chromosomes.
, NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5
, NADH ubiquinone oxidoreductase subunit B13
, NADH-ubiquinone oxidoreductase 13 kDa-B subunit
, complex I subunit B13
, complex I-13kD-B
, Complex I-13KD-B
, NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 5, 13kDa
, complex I 13kDa subunit B
, type I dehydrogenase
, ubiquinone reductase