Mitochondrial dysfunction in ALS

Mauro Cozzolino, Maria Teresa Carrì

Research output: Contribution to journalArticle

Abstract

In the present article, we review the many facets of mitochondrial dysfunction in amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease due to loss of upper motor neurons in cerebral cortex and lower motor neurons in brainstem and spinal cord. Accumulating evidence from recent studies suggests that the many, interconnected facets of mitochondrial dysfunction may play a more significant role in the etiopathogenesis of this disorder than previously thought. This notion stems from our expanding knowledge of the complex physiology of mitochondria and of alteration of their properties that might confer an intrinsic susceptibility to long-lived, post-mitotic motor neurons to energy deficit, calcium mishandling and oxidative stress.The wealth of evidence implicating mitochondrial dysfunction as a major event in the pathology of ALS has prompted new studies aimed to the development of new mitochondria-targeted therapies. However, it is now clear that drugs targeting more than one aspect of mitochondrial dysfunction are needed to fight this devastating disease.

Original languageEnglish
Pages (from-to)54-66
Number of pages13
JournalProgress in Neurobiology
Volume97
Issue number2
DOIs
Publication statusPublished - May 2012

Fingerprint

Amyotrophic Lateral Sclerosis
Motor Neurons
Mitochondria
Drug Delivery Systems
Neurodegenerative Diseases
Cerebral Cortex
Brain Stem
Spinal Cord
Oxidative Stress
Pathology
Calcium
Therapeutics

Keywords

  • Amyotrophic lateral sclerosis
  • Mitochondria
  • Motor neuron
  • SOD1

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Mitochondrial dysfunction in ALS. / Cozzolino, Mauro; Carrì, Maria Teresa.

In: Progress in Neurobiology, Vol. 97, No. 2, 05.2012, p. 54-66.

Research output: Contribution to journalArticle

Cozzolino, Mauro ; Carrì, Maria Teresa. / Mitochondrial dysfunction in ALS. In: Progress in Neurobiology. 2012 ; Vol. 97, No. 2. pp. 54-66.
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