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Nanoscopic quantification of sub-mitochondrial morphology, mitophagy and mitochondrial dynamics in living cells derived from patients with mitochondrial diseases

Authors :
Weiwei Zou
Qixin Chen
Jesse Slone
Li Yang
Xiaoting Lou
Jiajie Diao
Taosheng Huang
Source :
Journal of Nanobiotechnology, Vol 19, Iss 1, Pp 1-10 (2021)
Publication Year :
2021
Publisher :
BMC, 2021.

Abstract

Abstract SLC25A46 mutations have been found to lead to mitochondrial hyper-fusion and reduced mitochondrial respiratory function, which results in optic atrophy, cerebellar atrophy, and other clinical symptoms of mitochondrial disease. However, it is generally believed that mitochondrial fusion is attributable to increased mitochondrial oxidative phosphorylation (OXPHOS), which is inconsistent with the decreased OXPHOS of highly-fused mitochondria observed in previous studies. In this paper, we have used the live-cell nanoscope to observe and quantify the structure of mitochondrial cristae, and the behavior of mitochondria and lysosomes in patient-derived SLC25A46 mutant fibroblasts. The results show that the cristae have been markedly damaged in the mutant fibroblasts, but there is no corresponding increase in mitophagy. This study suggests that severely damaged mitochondrial cristae might be the predominant cause of reduced OXPHOS in SLC25A46 mutant fibroblasts. This study demonstrates the utility of nanoscope-based imaging for realizing the sub-mitochondrial morphology, mitophagy and mitochondrial dynamics in living cells, which may be particularly valuable for the quick evaluation of pathogenesis of mitochondrial morphological abnormalities.

Details

Language :
English
ISSN :
14773155
Volume :
19
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Journal of Nanobiotechnology
Publication Type :
Academic Journal
Accession number :
edsdoj.39682b776d684038999e0fdedfba589d
Document Type :
article
Full Text :
https://doi.org/10.1186/s12951-021-00882-9