Mitochondrial transcription termination factor 1 (MTERF1)

Mitochondrial transcription termination factor 1 (MTERF1) is a DNA-binding protein located in mitochondria, functioning as a key regulator of mitochondrial gene expression by mediating the sequence-specific termination of transcription within the mitochondrial genome[1][2][3][4][5][6]. MTERF1 binds specifically to a region adjacent to the mitochondrial 16S rRNA gene within the tRNA(Leu(UUR)) gene, where it induces DNA structural changes such as nucleotide base flipping, stabilizing its binding and enforcing directional transcriptional termination[1][2][3][4]. This precise halting of transcription allows for the proper expression balance of mitochondrial ribosomal RNAs and tRNAs[1][4][6]. MTERF1 also participates in modulating mitochondrial DNA replication, likely by acting as a barrier to replication fork progression, thus coordinating transcription and replication to maintain mitochondrial genome integrity[1][3][4]. Mutations that impair MTERF1's DNA-binding or base flipping functions are linked to mitochondrial diseases and may contribute to conditions like neuropathy, cardiomyopathy, cancer susceptibility, and altered cell proliferation, especially under stress such as hypoxia[1][3][4]. MTERF1 does not currently have any approved therapeutic drugs targeting it, nor established mechanisms of action, biomarkers, or safety concerns in clinical therapies, but its disruption is implicated in several mitochondrial dysfunctions[1][3][4][6].

Other names

MTERFmTERFmTERF1Transcription termination factor 1, mitochondrialTranscription termination factor, mitochondrial