Impaired Mitochondrial Morphology and Functionality in <i>Lonp1</i><sup>wt/−</sup> Mice

Impaired Mitochondrial Morphology and Functionality in <i>Lonp1</i><sup>wt/−</sup> Mice

LONP1 is a nuclear-encoded mitochondrial protease crucial for organelle homeostasis; mutations of <i>LONP1</i> have been associated with Cerebral, Ocular, Dental, Auricular, and Skeletal anomalies (CODAS) syndrome. To clarify the role of LONP1 in vivo, we generated a mouse model in which...

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Bibliographic Details
Main Authors: Anna De Gaetano, Lara Gibellini, Elena Bianchini, Rebecca Borella, Sara De Biasi, Milena Nasi, Federica Boraldi, Andrea Cossarizza, Marcello Pinti
Format: Article
Language:English
Published: MDPI AG 2020-06-01
Series:Journal of Clinical Medicine
Subjects:
Lon protease
CODAS
mitochondria
mouse model
enterocytes
mouse embryonic fibroblasts
Online Access:https://www.mdpi.com/2077-0383/9/6/1783

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