Regulation of a minimal transcriptome by repeat domain proteins.

Abstract Repeat proteins regulate the expression of the mammalian mitochondrial genome at the level of transcription, processing, maturation, and translation. Defects in the regulation of mitochondrial gene expression due to mutations in genes encoding repeat proteins can lead to mitochondrial dysfunction and disease, however the molecular mechanisms that regulate mitochondrial gene expression and how defects in these processes cause disease still remains poorly understood. Recently solved crystal structures, characterisation of the new genetic models, and use of RNA sequencing (RNA-Seq) technologies have greatly expanded our current understanding of mitochondrial repeat proteins and biology.