Bioenergetic markers in skin fibroblasts of sporadic amyotrophic lateral sclerosis and progressive lateral sclerosis patients. Academic Article uri icon

Overview

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.

publication date

  • August 21, 2014

Research

keywords

  • Amyotrophic Lateral Sclerosis
  • Energy Metabolism
  • Fibroblasts
  • Motor Neuron Disease
  • Skin

Identity

PubMed Central ID

  • PMC4192005

Scopus Document Identifier

  • 84907817312

Digital Object Identifier (DOI)

  • 10.1002/ana.24244

PubMed ID

  • 25090982

Additional Document Info

volume

  • 76

issue

  • 4