Roberta Filograna

Apr 28, 2024 | nature.com | Roberta Filograna |Markus Ringnér |Sara Riggare |Per Svenningsson |Michela Barbaro

AbstractLoss-of-function variants in the PRKN gene encoding the ubiquitin E3 ligase PARKIN cause autosomal recessive early-onset Parkinson’s disease (PD). Extensive in vitro and in vivo studies have reported that PARKIN is involved in multiple pathways of mitochondrial quality control, including mitochondrial degradation and biogenesis. However, these findings are surrounded by substantial controversy due to conflicting experimental data.

Aug 25, 2023 | cell.com | Johannes Hevler |Biomolecular Mass Spectrometry |Roberta Filograna

Highlights • Three amino acids (EED) in UQCRC1 of complex III2 control respirasome stability • Homozygous Uqcrc1delEED knockin mice have drastically reduced respirasome levels • Profound depletion of respirasomes does not impair oxidative phosphorylation • Mice with a profound depletion of respirasomes have apparently normal physiology Summary The mammalian respiratory chain complexes I, III2, and IV (CI, CIII2, and CIV) are critical for cellular bioenergetics and form a stable assembly, the...