Expert Reviews in Molecular Medicine

Table of Contents - Volume 12 - 2010  

Review Article

Mitochondrial quality control and neurological disease: an emerging connection

Inês Pimenta de Castroa1a2a3, L. Miguel Martinsa1 c1 and Roberta Tufia1

a1 Cell Death Regulation Laboratory, MRC Toxicology Unit, Lancaster Road, Leicester LE1 9HN, UK.

a2 Cancer Biology Group, IPATIMUP- Institute of Molecular Pathology and Immunology of the University of Porto, University of Porto, Portugal.

a3 Department of Microbiology, Faculty of Pharmacy, University of Porto, Portugal.

Abstract

The human brain is a highly complex organ with remarkable energy demands. Although it represents only 2% of the total body weight, it accounts for 20% of all oxygen consumption, reflecting its high rate of metabolic activity. Mitochondria have a crucial role in the supply of energy to the brain. Consequently, their deterioration can have important detrimental consequences on the function and plasticity of neurons, and is thought to have a pivotal role in ageing and in the pathogenesis of several neurological disorders. Owing to their inherent physiological functions, mitochondria are subjected to particularly high levels of stress and have evolved specific molecular quality-control mechanisms to maintain the mitochondrial components. Here, we review some of the most recent advances in the understanding of mitochondrial stress-control pathways, with a particular focus on how defects in such pathways might contribute to neurodegenerative disease.

Correspondence:

c1 Corresponding author: L. Miguel Martins, Cell Death Regulation Laboratory, MRC Toxicology Unit, University of Leicester, Hodgkin Building, Lancaster Road, Leicester LE1 9HN, UK. E-mail: lmm24@le.ac.uk

Metrics