Maria Miranda

TL;DR: Results showed that mitoTALENs, when expressed in affected tissues, could revert disease-related phenotypes in mice and reduce mutation burden and correct biochemical defects in a mouse model of mitochondrial disease.

TL;DR: Very unexpectedly, a new response pathway to OXPHOS dysfunction is identified in which the intra-mitochondrial synthesis of coenzyme Q (ubiquinone, Q) and Q levels are profoundly decreased, pointing towards novel possibilities for therapy.

TL;DR: In vivo evidence is presented that POLRMT has a key regulatory role in the replication of mammalian mtDNA and is part of a transcriptional mechanism that provides a switch between primer formation for mtDNA replication and mitochondrial gene expression.

TL;DR: Tefm is essential for mouse embryogenesis and that levels of promoter‐distal mitochondrial transcripts are drastically reduced in conditional Tefm‐knockout hearts, and loss of TEFM affects RNA processing in mammalian mitochondria.

TL;DR: It is reported that analysis of Polrmt gene expression, subcellular fractionation and fluorescence microscopy do not support the existence of a nuclear POLRMT isoform in mouse and human cells, and that conditional knockout of PolRMT does not affect expression of the nuclear genes previously reported to be transcribed by spRNAP-IV.