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Oxidative Phosphorylation Disease Diagnosis [PDF]

Seminars in Neurology, 1999
ABSTRACT: Although the mtDNA encodes only 13 polypeptide subunits of the OXPHOS enzymes, approximately 1,000 proteins are estimated to be necessary for proper OXPHOS function. Over the past 10 years a wide variety of adult and pediatric OXPHOS diseases were found to be caused by or associated with mitochondrial DNA (mtDNA) mutations and nuclear DNA ...
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The genetics and pathology of oxidative phosphorylation

Nature Reviews Genetics, 2001
The mitochondrial oxidative phosphorylation (OXPHOS) system is the final biochemical pathway in the production of ATP. The OXPHOS system consists of five multiprotein complexes, the individual subunits of which are encoded either by the mitochondrial or by the nuclear genome.
Smeitink, J.A.M., Heuvel, L.P.W.J. van den, Mauro, S. Di   +2 more
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Non-phosphorylated intermediates in oxidative phosphorylation

Biochimica et Biophysica Acta, 1963
Abstract 1. 1. This paper is concerned with the study of the formation of non-phosphorylated intermediates in digitonin particles prepared from rat-liver mitochondria. 2. 2. Digitonin particles were preincubated with either β-hydroxybutyrate, sucinate or ferrocytochrome c for a short time at 20°.
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Evidence of a phosphorylated intermediate in mitochondrial oxidative phosphorylation

Biochemical and Biophysical Research Communications, 1970
Abstract The effects of aurovertin and oligomycin on the rates of oxidative phosphorylation and arsenate-stimulated respiration have been studied over a wide range of concentrations. It has been found that both arsenate and phosphate can react with an energy-rich intermediate of oxidative phosphorylation even when reaction with ADP is prevented by ...
Jui H. Wang, Richard L. Cross
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Oxidative Phosphorylation

Annual Review of Biochemistry, 1962
A L, LEHNINGER, C L, WADKINS
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Cytochromes and Oxidative Phosphorylation

1970
The electron transport systems of brain include mitochondrial and nonmitochondrial bound cytochromes. The nonmitochondrial (microsomal) systems are involved in the oxidation of NAD(P)H, with eventual transfer of electrons to some acceptor either natural or artificial.
Cyril L. Moore, Paula M. Strasberg
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Oxidative Phosphorylation

2015
: The electron transport chain (ETC) is the major consumer of O2 in mammalian cells. The ETC passes electrons from NADH and FADH2 to protein complexes and mobile electron carriers. Coenzyme Q (CoQ) and cytochrome c (Cyt c) are mobile electron carriers in the ETC, and O2 is the final electron recipient.
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Evidence for a phosphorylated intermediate in oxidative phosphorylation

Biochemical and Biophysical Research Communications, 1962
D.E. Griffiths, R.A. Chaplain
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OXIDATIVE PHOSPHORYLATION

Australian Journal of Experimental Biology and Medical Science, 1958
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Oxidative Phosphorylation and Photophosphorylation

Annual Review of Biochemistry, 1977
Peter Mitchell, Efraim Racker, E. C. Slater, Lars Ernster, Britton Chance, Paul D. Boyer   +5 more
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