Acta Neuropathologica

, Volume 123, Issue 2, pp 157–171

Neurodegeneration as a consequence of failed mitochondrial maintenance


DOI: 10.1007/s00401-011-0921-0

Cite this article as:
Karbowski, M. & Neutzner, A. Acta Neuropathol (2012) 123: 157. doi:10.1007/s00401-011-0921-0


Maintaining the functional integrity of mitochondria is pivotal for cellular survival. It appears that neuronal homeostasis depends on high-fidelity mitochondria, in particular. Consequently, mitochondrial dysfunction is a fundamental problem associated with a significant number of neurological diseases, including Parkinson’s disease (PD), Huntington’s disease (HD), Alzheimer’s disease (AD), amyotrophic lateral sclerosis (ALS) and various peripheral neuropathies, as well as the normal aging process. To ensure optimal mitochondrial function, diverse, evolutionarily conserved mitochondrial quality control mechanisms are in place, including the scavenging of toxic reactive oxygen species (ROS) and degradation of damaged mitochondrial proteins, but also turnover of whole organelles. In this review we will discuss various mitochondria-associated conditions, focusing on the role of protein turnover in mitochondrial maintenance with special emphasis on neurodegenerative disorders.

Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  1. 1.Center for Biomedical Engineering and Technology and Department of Biochemistry and Molecular BiologyUniversity of Maryland School of MedicineBaltimoreUSA
  2. 2.Department of BiomedicineUniversity of BaselBaselSwitzerland
  3. 3.Department of OphthalmologyUniversity of BaselBaselSwitzerland

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