Also transcribed are the two rRNAs and 22 tRNAs required for mitochondrial translation. This process is accomplished, with the help of several accessory proteins, by the human mitochondrial RNA polymerase (POLRMT, also known as h-mtRNAP), a nuclear-encoded single-subunit DNA-dependent RNA polymerase (DdRp or RNAP) that is distantly related to ...

Sep 6, 2018 · The crystal structure of human mitochondrial RNA polymerase demonstrates a resemblance to phage RNAPs and highlights differences that may explain factor dependence of mtRNAP for initiation.

Sep 25, 2011 · Here we report the X-ray structure of human mtRNAP at 2.5 Å resolution, which reveals a T7-like catalytic carboxy-terminal domain, an amino-terminal domain that remotely resembles the T7...

Oct 2, 2023 · Mitochondrial transcription is performed by the single-subunit, mitochondrial RNA polymerase (POLRMT), which is related to the T7 bacteriophage RNA polymerase (T7 RNAP) 6.

TFAM and mitochondrial RNA processing enzymes are target genes of NRF1, and TFBM1 and TFBM2 were also recently demonstrated to be their target. These two proteins enhance mtDNA transcription in the presence of TFAM and mitochondrial RNA polymerase.

Among other nucleoid core factors, one can distinguish mtDNA polymerase γ (POLG), mitochondrial single-stranded DNA-binding protein (mtSSB), the NAD-dependent protein deacetylase sirtuin-1 (SIRT1), Twinkle helicase, and mitochondrial RNA …

The human mitochondrial RNA polymerase (h-mtRNAP) serves as both the transcriptase for expression and the primase for replication of mitochondrial DNA. As such, the enzyme is of fundamental importance to cellular energy metabolism, and defects in its function may be related to human disease states.

Sep 1, 2012 · The human mitochondrial RNA polymerase (POLRMT) transcribes the mitochondrial genome with the help of accessory factors. POLRMT is evolutionarily related to bacteriophage T7 RNAP. Understanding properties of POLRMT is central to comprehending the mechanism of human mitochondrial transcription.

Sep 25, 2011 · Here we report the X-ray structure of human mtRNAP at 2.5 Å resolution, which reveals a T7-like catalytic carboxy-terminal domain, an amino-terminal domain that remotely resembles the T7 promoter-binding domain, a novel pentatricopeptide repeat domain, and a flexible N-terminal extension.

Sep 1, 2012 · Mitochondrial polyadenylation regulates RNA function and metabolism. Mitochondrial polyadenylation occurs in higher eukaryotes. Human mitochondrial poly(A) polymerase (PAPD1) is a noncanonical PAP. The structure of PAPD1 provides molecular insights into its catalysis.