Article
Increased longevity and refractoriness to Ca(2+)-dependent neurodegeneration in Surf1 knockout mice.
Human molecular genetics - 15 Feb 2007
Dell'agnello Carlotta, Leo Sara, Agostino Alessandro, Szabadkai György, Tiveron Cecilia, Zulian Alessandra, Prelle Alessandro, Roubertoux Pierre, Rizzuto Rosario, Zeviani Massimo
Abstract excerpt
Leigh syndrome associated with cytochrome c oxidase (COX) deficiency is a mitochondrial disorder usually caused by mutations of SURF1, a gene encoding a putative COX assembly factor. We present here a Surf1-/- recombinant mouse obtained by inserting a loxP sequence in the open reading frame of th...
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