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Mitochondrial Transport and Turnover in the Pathogenesis of Amyotrophic Lateral Sclerosis

Feil Family Brain and Mind Research Institute, Weill Cornell Medicine, 407 East 61st Street, New York, NY 10065, USA
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Biology 2019, 8(2), 36;
Received: 16 December 2018 / Revised: 20 January 2019 / Accepted: 3 February 2019 / Published: 11 May 2019
(This article belongs to the Special Issue Mitochondrial Dysfunction in Ageing and Diseases of Ageing)
PDF [648 KB, uploaded 11 May 2019]


Neurons are high-energy consuming cells, heavily dependent on mitochondria for ATP generation and calcium buffering. These mitochondrial functions are particularly critical at specific cellular sites, where ionic currents impose a large energetic burden, such as at synapses. The highly polarized nature of neurons, with extremely large axoplasm relative to the cell body, requires mitochondria to be efficiently transported along microtubules to reach distant sites. Furthermore, neurons are post-mitotic cells that need to maintain pools of healthy mitochondria throughout their lifespan. Hence, mitochondrial transport and turnover are essential processes for neuronal survival and function. In neurodegenerative diseases, the maintenance of a healthy mitochondrial network is often compromised. Numerous lines of evidence indicate that mitochondrial impairment contributes to neuronal demise in a variety of neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), where degeneration of motor neurons causes a fatal muscle paralysis. Dysfunctional mitochondria accumulate in motor neurons affected by genetic or sporadic forms of ALS, strongly suggesting that the inability to maintain a healthy pool of mitochondria plays a pathophysiological role in the disease. This article critically reviews current hypotheses on mitochondrial involvement in the pathogenesis of ALS, focusing on the alterations of mitochondrial axonal transport and turnover in motor neurons. View Full-Text
Keywords: mitochondria; ALS; axonal transport; mitophagy; SOD1; Miro1; PINK1; Parkin mitochondria; ALS; axonal transport; mitophagy; SOD1; Miro1; PINK1; Parkin

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Granatiero, V.; Manfredi, G. Mitochondrial Transport and Turnover in the Pathogenesis of Amyotrophic Lateral Sclerosis. Biology 2019, 8, 36.

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