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Article

Muscle 3243A-->G mutation load and capacity of the mitochondrial energy-generating system.

Annals of neurology - 1 Apr 2008

Janssen Antoon J M, Schuelke Markus, Smeitink Jan A M, Trijbels Frans J M, Sengers Rob C A, Lucke Barbara, Wintjes Liesbeth T M, Morava Eva, van Engelen Baziel G M, Smits Bart W, Hol Frans A, Siers Marloes H, Ter Laak Henk, van der Knaap Marjo S, Van Spronsen Francjan J, Rodenburg Richard J T, van den Heuvel Lambert P

Abstract excerpt

OBJECTIVE: The mitochondrial energy-generating system (MEGS) encompasses the mitochondrial enzymatic reactions from oxidation of pyruvate to the export of adenosine triphosphate. It is investigated in intact muscle mitochondria by measuring the pyruvate oxidation and adenosine triphosphate production rates, which we refer to as the "MEGS capacity." Currently, little is known about MEGS pathology in patients with...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 18306232
DOI: 10.1002/ana.21328

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