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Advances in Mitochondrial Medicine pp 269–286Cite as

Mitochondria in Neurodegeneration

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Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 942))

Abstract

Many neurodegenerative diseases demonstrate abnormal mitochondrial morphology and biochemical dysfunction. Alterations are often systemic rather than brain-limited. Mitochondrial dysfunction may arise as a consequence of abnormal mitochondrial DNA, mutated nuclear proteins that interact directly or indirectly with mitochondria, or through unknown causes. In most cases it is unclear where mitochondria sit in relation to the overall disease cascades that ultimately causes neuronal dysfunction and death, and there is still controversy regarding the question of whether mitochondrial dysfunction is a necessary step in neurodegeneration. In this chapter we highlight and catalogue mitochondrial perturbations in some of the major neurodegenerative diseases including Alzheimer’s disease (AD), Parkinson’s disease (PD), amyotrophic lateral sclerosis (ALS), and Huntington’s disease (HD). We consider data that suggest mitochondria may be critically involved in neurodegenerative disease neurodegeneration cascades.

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  1. Departments of Neurology, Molecular and Integrative Physiology, University of Kansas School of Medicine, MSN 2012, Landon Center on Aging, 3901 Rainbow Blvd, Kansas City, KS, 66209, USA

    E Lezi & Russell H. Swerdlow MD

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  1. School of Medicine, Institute of Biochemistry and Clinical B, Catholic University, Largo A. Gemelli 8, Rome, 00168, Italy

    Roberto Scatena

  2. School of Medicine, Institute of Biochemistry and Clinical B, Catholic University, Largo A. Gemelli 8, Rome, 00168, Italy

    Patrizia Bottoni

  3. School of Medicine, Institute of Biochemistry and Clinical B, Catholic University, Largo A. Gemelli 8, Rome, 00168, Italy

    Bruno Giardina

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Lezi, E., Swerdlow, R.H. (2012). Mitochondria in Neurodegeneration. In: Scatena, R., Bottoni, P., Giardina, B. (eds) Advances in Mitochondrial Medicine. Advances in Experimental Medicine and Biology, vol 942. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-2869-1_12

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