Aleksandra Trifunovic

TL;DR: In this paper, an intricate interplay between three transcription factors regulating the mitochondrial stress response: CHOP, C/EBPβ, and ATF4 was reported, showing that CHOP acts as a rheostat that attenuates prolonged ISR, prevents unfavorable metabolic alterations, and postpones the onset of mitochondrial cardiomyopathy.

TL;DR: The role of different repair, replication and maintenance mechanisms that contribute to mtDNA integrity and mutagenesis will be discussed in details in this article.

TL;DR: A critical role for CLPP is demonstrated in different metabolic stress conditions such as high‐fat diet feeding and cold exposure providing tools to understand pathologies with deregulated Clpp expression and novel insights into therapeutic approaches against metabolic dysfunctions linked to mitochondrial diseases.

TL;DR: The symptomatology of WARS2 is substantially extended by presenting a patient with severe infantile‐onset leukoencephalopathy, profound intellectual disability, spastic quadriplegia, epilepsy, microcephaly, short stature, failure to thrive, cerebral atrophy, and periventricular white matter abnormalities.

TL;DR: The data suggest that aging is associated with a progressive decline in β-cell mitochondrial function that negatively impacts on the fine tuning of Ca2+ dynamics, and this is conceptually important since it emphasizes that even relatively modest changes inβ-cell signal transduction over time lead to compromised insulin release and a diabetic phenotype.