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- Tribouillard-Tanvier, Déborah, et al. Show all 6 Authors
- Biochimica et biophysica acta 2019 v.1860 no.7 pp. 562-572
- H+/K+-exchanging ATPase; H-transporting ATP synthase; Saccharomyces cerevisiae; adenosine triphosphate; arginine; childhood; hydrophilicity; lysine; methionine; mitochondria; mitochondrial genes; mitochondrial membrane; mutants; mutation; nervous system diseases; protons; tryptophan; yeasts
- ... Dozens of pathogenic mutations have been localized in the mitochondrial gene (MT-ATP6) that encodes the subunit a of ATP synthase. The subunit a together with a ring of identical subunits c moves protons across the mitochondrial inner membrane coupled to rotation of the subunit c-ring and ATP synthesis. One of these mutations, m.8851T>C, has been associated with bilateral striatal lesions of child ...
- Tribouillard-Tanvier, Déborah, et al. Show all 5 Authors
- Biochimica et biophysica acta 2019 v.1860 no.1 pp. 52-59
- H+/K+-exchanging ATPase; H-transporting ATP synthase; Saccharomyces cerevisiae; adenosine triphosphate; arginine; electrons; electrostatic interactions; genes; humans; hydrophilicity; hydrophobicity; inheritance (genetics); leucine; mitochondria; mutation; oxygen; peripheral nervous system diseases; protons; retinitis pigmentosa; yeasts
- ... Protons are transported from the mitochondrial matrix to the intermembrane space of mitochondria during the transfer of electrons to oxygen and shuttled back to the matrix by the a subunit and a ring of identical c subunits across the membrane domain (FO) of ATP synthase, which is coupled to ATP synthesis. A mutation (m.9176 T > G) of the mitochondrial ATP6 gene that replaces an universally conser ...