Mitochondrial Quality Control: a Matter of Life and Death for Neurons

Overview

Journal EMBO J

Specialties Biotechnology
Molecular Biology

Date 2012 Feb 23

PMID 22354038

Citations 205

Authors

Elena I Rugarli

Thomas Langer

Affiliations

Soon will be listed here.

Abstract

Neuronal survival critically depends on the integrity and functionality of mitochondria. A hierarchical system of cellular surveillance mechanisms protects mitochondria against stress, monitors mitochondrial damage and ensures the selective removal of dysfunctional mitochondrial proteins or organelles. Mitochondrial proteases emerge as central regulators that coordinate different quality control (QC) pathways within an interconnected network of mechanisms. A failure of this system causes neuronal loss in a steadily increasing number of neurodegenerative disorders, which include Parkinson's disease, spinocerebellar ataxia, spastic paraplegia and peripheral neuropathies. Here, we will discuss the role of the mitochondrial QC network for neuronal survival and neurodegeneration.

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