Article
Optic nerve histopathology in a case of Wolfram Syndrome: a mitochondrial pattern of axonal loss.
Mitochondrion - 1 Nov 2013
Ross-Cisneros Fred N, Pan Billy X, Silva Ruwan A, Miller Neil R, Albini Thomas A, Tranebjaerg Lisbeth, Rendtorff Nanna D, Lodahl Marianne, Moraes-Filho Milton N, Moraes Milton N, Salomao Solange R, Berezovsky Adriana, Belfort Rubens, Carelli Valerio, Sadun Alfredo A
Abstract excerpt
Mitochondrial dysfunction in Wolfram Syndrome (WS) is controversial and optic neuropathy, a cardinal clinical manifestation, is poorly characterized. We here describe the histopathological features in postmortem retinas and optic nerves (ONs) from one patient with WS, testing the hypothesis that mitochondrial dysfunction underlies the pathology. Eyes and retrobulbar ONs were obtained at autopsy from a WS patient,...
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