tRNA-nucleotidyltransferase 1, is an enzyme that in humans is encoded by the TRNT1 gene. (wikipedia.org)
This CCA deactivation is reversible and quickly repairable by the CCA-adding enzyme [ATP(CTP):tRNA nucleotidyltransferase]. (prolekarniky.cz)
Protein2
PNPT1 (polyribonucleotide nucleotidyltransferase 1, mitochondirla) is a RNA-binding protein implicated in numerous RNA metabolic processes. (thermofisher.com)
CSAs involve mutations in 1 of 3 mitochondrial pathways: heme synthesis, iron-sulfur cluster biogenesis, and protein synthesis. (medscape.com)
Polymerase1
Chen CC, Simard MJ, Tabara H, Brownell DR, McCollough JA, Mello CC. A member of the polymerase beta nucleotidyltransferase superfamily is required for RNA interference in C. elegans. (umassmed.edu)
TRNA3
A eubacterial origin for the human tRNA nucleotidyltransferase? (wikipedia.org)
2015) The 3' addition of CCA to mitochondrial tRNASer(AGY) is specifically impaired in patients with mutations in the tRNA nucleotidyl transferase TRNT1. (rnacentral.org)
Predicted to enable nucleotidyltransferase activity and tRNA binding activity. (nih.gov)
Translocases1
A class of nucleotide translocases found abundantly in mitochondria that function as integral components of the inner mitochondrial membrane. (nih.gov)
Defects2
This is the most common of the hereditary sideroblastic anemias, followed by mitochondrial transporter defects such as SLC25A38 gene mutation discussed below. (medscape.com)
T can be summarized as follows: This mutation firstly loses the appropriate base pair interaction, from Watson-Crick to T-G mismatching, in the anticodon-stem of MT-TW gene and induces defects in mitochondrial tRNATrp-associated translation machinery most likely due to inadequate anticodon recognition of mitochondrial tryptophan by its altered conformation [1]. (rnacentral.org)
Gene1
Predominant cellular mitochondrial dysfunction in the TOP3A gene-caused Bloom syndrome-like disorder. (nih.gov)
MEMBRANE1
Further mechanistic study has shown that the mitochondrial oxidative phosphorylation (OXPHOS), membrane potential, and ATP production were significantly decreased two days after the overexpression of S and ORF-9B subunits, while S subunits induced higher level of reactive oxygen species (ROS). (bvsalud.org)
Human1
2012) The human mitochondrial transcriptome Cell 146(4):645-658. (rnacentral.org)
Complex1
PNPT1 is a component of the mitochondrial degradosome (mtEXO) complex that degrades 3' overhang double-stranded RNA with a 3'-to-5' directionality in an ATP-dependent manner. (thermofisher.com)
A pathogenic variant causing exon skipping was identified in the gene encoding Polyribonucleotide Nucleotidyltransferase PNPase 1 (PNPT1) located in the SCA25 linkage interval. (nih.gov)
Encodes2
G) that encodes the mitochondrial polynucleotide phosphorylase (PNPase). (nih.gov)
This gene encodes a mitochondrial DNA-directed RNA polymerase. (nih.gov)
PNPase1
PNPase is predominantly localized in the mitochondrial intermembrane space and is implicated in RNA targeting to human mitochondria. (nih.gov)
Mammalian1
There, she combines stable isotope pulse chase labeling with mass spectrometry to investigate mitochondrial long-lived proteome in mammalian brains. (nih.gov)
TRNAs1
Balasubramaniam M, Reis RJS, Ayyadevara S, Wang X, Ganne A, Khaidakov M. Involvement of tRNAs in replication of human mitochondrial DNA and modifying effects of telomerase. (uams.edu)
Transcription1
DNA-dependent RNA polymerase catalyzes the transcription of mitochondrial DNA into RNA using the four ribonucleoside triphosphates as substrates. (nih.gov)
Proteins1
Many mitochondrial proteins form groups (complexes) that carry out the reactions that produce energy. (medlineplus.gov)
Mutations2
Structural insight into processive human mitochondrial DNA synthesis and disease-related polymerase mutations. (nih.gov)
Nous avons ainsi suivi, presque au jour le jour, le devenir de la composition du génome viral pour la relier à la descendance produite au cours du temps, en particulier sous la forme d'efflorescences où apparaît un véritable feu d'artifice de mutations virales. (academie-sciences.fr)
Replication1
The gene product is responsible for mitochondrial gene expression as well as for providing RNA primers for initiation of replication of the mitochondrial genome. (nih.gov)
Respiratory-chain1
Multiple-respiratory-chain deficiency represents an important cause of mitochondrial disorders. (nih.gov)
Catalyzes1
cGAS (Cyclic GMP-AMP synthase) is a nucleotidyltransferase that catalyzes the formation of cyclic GMP-AMP (cGAMP) from ATP and GTP and plays a key role in innate immunity. (thermofisher.com)
Transporter1
This is the most common of the hereditary sideroblastic anemias, followed by mitochondrial transporter defects such as SLC25A38 gene mutation discussed below. (medscape.com)
Although this polypeptide has the same function as the three nuclear DNA-directed RNA polymerases, it is more closely related to RNA polymerases of phage and mitochondrial polymerases of lower eukaryotes. (nih.gov)