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Mutant mitochondrial elongation factor G1 and combined oxidative phosphorylation deficiency

Article Abstract:

The two siblings with a severe defect in mitochondrial translation, reduced levels of oxidative phosphorylation complexes containing mitochondrial DNA (mtDNA) encoded subunits, and progressive hepatoencephalopathy are investigated. The defective gene to a region on chromosome 3q containing elongation factor G1, which encodes a mitochondrial translation factor is outlined.

Author: Newbold, Robert F., Coenem, Marieke J.H., Antonicka, Hana, Trijbels, Frans J.M.F., Ugalde, Cristina, Heuvel, Lambert P. van den, Sasarman, Florin, Shoubridge, Eric A., Smeitink, Jan A.M., Rossi, Rainer, Heister, J.M.F. Angelien
Publisher: Massachusetts Medical Society
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 2004
United Kingdom, Phosphorylation, Mitochondrial diseases

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Electron-transfer processes of cytochrome c at interfaces. New insights by surface-enhanced resonance Raman spectroscopy

Article Abstract:

The heme protein cytochrome c acts as an electron carrier at the mitochondriat membrane interface and exerts its function under the influence of strong electric fields. The physiologically relevant electric field strengths can effectively modulate the electron-transfer dynamics and induce conformational transitions.

Author: Hildebrandt, Peter, Murgida, Daniel H.
Publisher: Massachusetts Medical Society
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 2004
Electron transport, Cytochrome c, Atomic properties, Hemoproteins

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