Mitochondrial transcription rescue factor 1 (MTRES1)

Mitochondrial transcription rescue factor 1 (MTRES1) is a mitochondrial RNA-binding protein encoded in the nucleus and localized to the mitochondrial matrix[2][4]. It plays a critical role in maintaining proper levels of mitochondrial RNA, especially during cellular stress that perturbs mitochondrial transcription[1][2][3][4]. MTRES1 is upregulated in response to diminished mitochondrial nucleic acid synthesis, functioning as a protective factor that acts at the transcriptional level through its direct RNA-binding activity, rather than altering RNA stability or mitochondrial DNA copy number[1][2][3][4]. MTRES1 associates with the mitochondrial RNA polymerase (POLRMT) and the transcription initiation factor TFAM, acting to rescue and restore mitochondrial transcript levels under stress conditions[1][2]. This factor is essential for sustaining mitochondrial gene expression and normal mitochondrial function, and genetic dysfunction in MTRES1 has been linked to certain mitochondrial disorders such as Optic atrophy 10 and Leigh disease[5]. No drugs or biomarkers are currently associated with MTRES1, and it is not generally considered a classic therapeutic target such as a receptor, enzyme, or transporter[5].

Other names

C6orf203HSPC230PRED31uncharacterized protein C6orf203