Ask about this productRelated genes to: MTRF1L antibody
- Gene:
- MTRF1L NIH gene
- Name:
- mitochondrial translational release factor 1 like
- Previous symbol:
- -
- Synonyms:
- HMRF1L, mtRF1a
- Chromosome:
- 6q25.2
- Locus Type:
- gene with protein product
- Date approved:
- 2003-05-12
- Date modifiied:
- 2016-10-05
Related products to: MTRF1L antibody
Related articles to: MTRF1L antibody
- Because mitochondria diverged from a bacterial ancestor during evolution, the mitochondrial protein synthesis system includes both mRNAs and translation factors with unique characteristics. However, the molecular mechanisms underlying translation termination, recycling, and quality control remain unclear. Here, via high-resolution mitochondrial Ribo-Seq and Disome-Seq, we reveal: the specificity of release factors for different kinds of stop codons; the role of mtRF1 in vertebrates, which do not have noncanonical stop codons in their main translons; the recycling-coupled translation of internal translons; and the rescue of mitoribosomes in the early elongation stage. mtRF1L recognizes all stop codons, whereas mtRF1 recognizes only AGA/AGG noncanonical stop codons. Additionally, mtRF1 terminates the translation of out-of-frame translons that end with AGA/AGG. We also found that mtRRF and mtIF3 are required for mitoribosome recycling on stop codons and for the reinitiation of internal translon translation. Mitoribosomes that stall at the start codons and/or at the early elongation phase are major substrates of the rescue factors ICT1, mtRF-R, and mtRES1. Moreover, HEMK1-mediated methylation of release factors enhances the termination reaction on stop codons. Our results provide insights into the mitoribosome dynamics that are associated with the completion of protein synthesis. - Source: PubMed
Publication date: 2026/07/21
Wakigawa TaiseiMito MariFang QiItoh YuzuruShinkai YoichiIwasaki Shintaro - The increasing prevalence of sarcopenia (SAR) has raised significant concerns in healthcare. Although mitochondrial dysfunction and immune disorders are recognized as risk factors, the interactions between them remain unclear. This study aims to identify potential diagnostic biomarkers associated with both phenotypes in the progression of SAR. Three transcriptional datasets were obtained from the GEO database. Gene set enrichment analysis (GSEA) was performed to explore the features of the training set, followed by filtering the differentially expressed genes (DEGs). Weighted gene co-expression network analysis was applied to select gene modules closely related to SAR. SAR-mitochondria-related DEGs were then determined by intersecting the DEGs, weighted gene co-expression network analysis, and mitochondrial-related genes from the MitoCarta3.0 database. Hub genes were further explored using LASSO and random forest machine learning algorithms. The regulatory molecules of these hub genes were predicted using the NetworkAnalyst database. Subsequently, receiver operating characteristic analysis was performed and the immune infiltration was analyzed using the CIBERSORT algorithm. A SAR model was established in C2C12 cells using d-galactose, and RT-qPCR experiments were performed for further validation. Gene set enrichment analysis results revealed that the training set genes are mainly enriched in mitochondrial function and energy metabolism. Through the machine learning methods, 5 hub genes were screened out from 32 SAR-Mito DEGs, namely MTRF1L, MICU1, DHTKD1, ACADM, and FHIT. A total of 42 transcription factors and 10 miRNAs strongly associated with the hub genes were detected. These hub genes demonstrated solid diagnostic potential in both training and validation sets. Furthermore, immune infiltration analysis indicated a significant reduction in neutrophil levels in SAR patients. The downregulation of the hub genes in d-galactose-induced C2C12 cells was confirmed. Collectively, our findings identified 5 potential biomarkers for the diagnosis and therapy of SAR and emphasized the interaction between mitochondrial function and the immune response in the development of this condition. - Source: PubMed
Yan HongyuLi YangLiu ZhongyuanZhou TianleLiu KaiXiong LingHuang Fei - Mitochondria have been linked with inflammatory colorectal cancer (CRC) development; however, the association between mitochondria-related genes (MRGs) and CRC remains unknown. - Source: PubMed
Zhu LiminHuang XiaoweiZhang FanYang JinzuXu Zhenye - The genetic code that specifies the identity of amino acids incorporated into proteins during protein synthesis is almost universally conserved. Mitochondrial genomes feature deviations from the standard genetic code, including the reassignment of two arginine codons to stop codons. The protein required for translation termination at these noncanonical stop codons to release the newly synthesized polypeptides is not currently known. In this study, we used gene editing and ribosomal profiling in combination with cryo-electron microscopy to establish that mitochondrial release factor 1 (mtRF1) detects noncanonical stop codons in human mitochondria by a previously unknown mechanism of codon recognition. We discovered that binding of mtRF1 to the decoding center of the ribosome stabilizes a highly unusual conformation in the messenger RNA in which the ribosomal RNA participates in specific recognition of the noncanonical stop codons. - Source: PubMed
Publication date: 2023/05/04
Saurer MartinLeibundgut MarcNadimpalli Hima PriyankaScaiola AlainSchönhut TanjaLee Richard GSiira Stefan JRackham OliverDreos RenéLenarčič TeaKummer EvaGatfield DavidFilipovska AleksandraBan Nenad - The mitochondrial translation machinery highly diverged from its bacterial counterpart. This includes deviation from the universal genetic code, with AGA and AGG codons lacking cognate tRNAs in human mitochondria. The locations of these codons at the end of COX1 and ND6 open reading frames, respectively, suggest they might function as stop codons. However, while the canonical stop codons UAA and UAG are known to be recognized by mtRF1a, the release mechanism at AGA and AGG codons remains a debated issue. Here, we show that upon the loss of another member of the mitochondrial release factor family, mtRF1, mitoribosomes accumulate specifically at AGA and AGG codons. Stalling of mitoribosomes alters COX1 transcript and protein levels, but not ND6 synthesis. In addition, using an in vitro reconstituted mitochondrial translation system, we demonstrate the specific peptide release activity of mtRF1 at the AGA and AGG codons. Together, our results reveal the role of mtRF1 in translation termination at non-canonical stop codons in mitochondria. - Source: PubMed
Publication date: 2023/01/03
Krüger AnnikaRemes CristinaShiriaev Dmitrii IgorevichLiu YongSpåhr HenrikWibom RolfAtanassov IlianNguyen Minh DucCooperman Barry SRorbach Joanna