Transcription elongation factor, mitochondrial (TEFM)

Transcription elongation factor, mitochondrial (TEFM) is a nuclear-encoded protein localized to the mitochondria, where it acts as a key component of the mitochondrial transcription machinery. TEFM interacts with the mitochondrial RNA polymerase (POLRMT) to increase its processivity, enabling the transcription of near genome-length polycistronic mitochondrial RNA necessary for gene expression and mtDNA replication. TEFM regulates both transcription elongation and, indirectly, the processing of mitochondrial transcripts, as well as the formation of replication primers. Loss or mutation of TEFM leads to impaired transcription elongation, a drastic reduction of promoter-distal mitochondrial transcripts, defects in RNA processing, a drop in mtDNA replication, and pronounced OXPHOS dysfunction. Pathogenic variants in TEFM cause mitochondrial respiratory chain deficiencies with a spectrum of clinical phenotypes, most notably childhood-onset mitochondrial myopathy and neuromuscular deficits[1][2][3].

Other names

TEFMC17orf42FLJ22729COXPD58transcription elongation factor of mitochondria

Not applicable—there are currently no drugs known to directly act on TEFM[1][2][3]