Impaired Mitochondrial Morphology and Functionality in Lonp1^wt/- Mice.

ABSTRACT: LONP1 is a nuclear-encoded mitochondrial protease crucial for organelle homeostasis; mutations of LONP1 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 Lonp1 was ablated. The homozygous Lonp^-/- mouse was not vital, while the heterozygous Lonp1^wt/- showed similar growth rate, weight, length, life-span and histologic features as wild type. Conversely, ultrastructural analysis of heterozygous enterocytes evidenced profound morphological alterations of mitochondria, which appeared increased in number, swollen and larger, with a lower complexity. Embryonic fibroblasts (MEFs) from Lonp1^wt/- mice showe

SUBMITTER: De Gaetano A 

PROVIDER: S-EPMC7355737 | biostudies-literature | 2020 Jun

REPOSITORIES: biostudies-literature