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Publication Detail

Title: Bioenergetic markers in skin fibroblasts of sporadic amyotrophic lateral sclerosis and progressive lateral sclerosis patients.

Authors: Kirk, Kathryne; Gennings, Chris; Hupf, Jonathan C; Tadesse, Saba; D'Aurelio, Marilena; Kawamata, Hibiki; Valsecchi, Federica; Mitsumoto, Hiroshi; ALS/PLS COSMOS Study Groups; Manfredi, Giovanni

Published In Ann Neurol, (2014 Oct)

Abstract: Energy metabolism could influence amyotrophic lateral sclerosis (ALS) and progressive lateral sclerosis (PLS) pathogenesis and the response to therapy. We developed a novel assay to simultaneously assess mitochondrial content and membrane potential in patients' skin fibroblasts. In ALS and PLS fibroblasts, membrane potential was increased and mitochondrial content decreased, relative to healthy controls. In ALS higher mitochondrial membrane potential correlated with age at diagnosis, and in PLS it correlated with disease severity. These unprecedented findings in ALS and PLS fibroblasts could shed new light onto disease pathogenesis and help in developing biomarkers to predict disease evolution and the individual response to therapy in motor neuron diseases.

PubMed ID: 25090982 Exiting the NIEHS site

MeSH Terms: No MeSH terms associated with this publication

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