Mechanisms of Mitochondrial Dysfunction in Lysosomal Storage Disorders: A Review

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ID: 114208
2020
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Abstract
Mitochondrial dysfunction is emerging as an important contributory factor to the pathophysiology of lysosomal storage disorders (LSDs). The cause of mitochondrial dysfunction in LSDs appears to be multifactorial, although impaired mitophagy and oxidative stress appear to be common inhibitory mechanisms shared amongst these heterogeneous disorders. Once impaired, dysfunctional mitochondria may impact upon the function of the lysosome by the generation of reactive oxygen species as well as depriving the lysosome of ATP which is required by the V-ATPase proton pump to maintain the acidity of the lumen. Given the reported evidence of mitochondrial dysfunction in LSDs together with the important symbiotic relationship between these two organelles, therapeutic strategies targeting both lysosome and mitochondrial dysfunction may be an important consideration in the treatment of LSDs. In this review we examine the putative mechanisms that may be responsible for mitochondrial dysfunction in reported LSDs which will be supplemented with morphological and clinical information.
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Authors Karolina M. Stepien;Federico Roncaroli;Nadia Turton;Christian J. Hendriksz;Mark Roberts;Robert A. Heaton;Iain Hargreaves;Stepien, Karolina M.;Roncaroli, Federico;Turton, Nadia;Hendriksz, Christian J.;Roberts, Mark;Heaton, Robert A.;Hargreaves, Iain;
Journal journal of clinical medicine
Year 2020
DOI 10.3390/jcm9082596
URL
https://www.mdpi.com/2077-0383/9/8/2596
Keywords
inflammation oxidative stress autophagy reactive oxygen species mitochondrial dysfunction lysosomal storage diseases mitophagy and cytokine

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