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Therapeutic outcome of patients with Lennox-Gastaut syndrome with mitochondrial respiratory chain complex I deficiency. [PDF]

Front Neurol
Na JH, Lee YM.
europepmc   +1 more source

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Respiratory Chain Supercomplexes

IUBMB Life, 2001
AbstractRespiratory chain supercomplexes have been isolated from mammalian and yeast mitochondria, and bacterial membranes. Functional roles of respiratory chain supercomplexes are catalytic enhancement, substrate channelling, and stabilization of complex I by complex III in mammalian cells.
Hermann Schägger, H Schägger
exaly   +3 more sources

The mitochondrial respiratory chain

2023
We present a brief review of the mitochondrial respiratory chain with emphasis on complexes I, III and IV, which contribute to the generation of protonmotive force across the inner mitochondrial membrane, and drive the synthesis of ATP by the process called oxidative phosphorylation.
Mårten Wikström, Cristina Pecorilla, Vivek Sharma   +2 more
openaire   +2 more sources

The mitochondrial respiratory chain

Essays in Biochemistry, 2010
In the present chapter, the structures and mechanisms of the major components of mammalian mitochondrial respiratory chains are reviewed. Particular emphasis is placed on the four protein complexes and their cofactors that catalyse the electron transfer pathway between oxidation of NADH and succinate and the reduction of oxygen to water.
Peter R, Rich, Amandine, Maréchal
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Respiratory chain deficiencies

2013
Mitochondrial functions are intimately associated with neurological symptoms. Various clinical and biological features are suggestive of energy depletion diseases, such as Leigh syndrome, Alpers syndrome, epilepsy (including myoclonic seizures and status epilepticus), stroke-like episodes, and acute cerebellar ataxia with high lactate peaks on magnetic
Pascale, Delonlay, Agnes, Rötig, Harvey B, Sarnat   +2 more
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The Respiratory Chain

2012
The mitochondrial electron transport respiratory chain of higher animals is described. The main principles of energy conservation in the form of electrochemical potential of hydrogen ions are presented. Structural and functional properties of NADH: quinone-oxidoreductase, quinol: cytochrome c-oxidoreductase, and cytochrome c oxidase (complexes I, II ...
Vladimir P. Skulachev, Alexander V. Bogachev, Felix O. Kasparinsky   +2 more
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Defective assembly of the respiratory chain

Acta Paediatrica, 2004
A functional respiratory chain is dependent on protein components encoded by both mtDNA and nuclear DNA. Isolated cytochrome c oxidase (COX) deficiency is often caused by mutations in nuclear genes regulating the assembly of the 13 protein subunits of this complex. The accompanying paper by Zeman and co‐workers reports that mutations in SCO2 are common
Esteitie, N., Larsson, N.
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Ceramide and the mitochondrial respiratory chain

Biochimie, 2014
Ceramide is a group of sphingolipids found in cell membranes, composed of a sphingoid base linked to a fatty acid of varying chain length. Initially regarded as purely structural components, this group of molecules is now recognized as a key signaling and regulatory elements in cell biology.
Aviram, Kogot-Levin, Ann, Saada
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Mitochondrial Respiratory Chain Complexes

2018
Mitochondria are the power stations of the eukaryotic cell, using the energy released by the oxidation of glucose and other sugars to produce ATP. Electrons are transferred from NADH, produced in the citric acid cycle in the mitochondrial matrix, to oxygen by a series of large protein complexes in the inner mitochondrial membrane, which create a ...
Sousa, J., D'Imprima, E., Vonck, J. ; https://orcid.org/0000-0001-5659-8863   +2 more
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Mitochondrial Respiratory-Chain Diseases

New England Journal of Medicine, 2003
The mitochondrial respiratory chain has the crucial function of supplying the cell with energy in the form of ATP. Mutations affecting this chain can arise in mitochondrial or nuclear DNA and cause diseases known as mitochondrial encephalomyopathies.
Salvatore, DiMauro, Eric A, Schon
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