Alternative titles; symbols
HGNC Approved Gene Symbol: MTRES1
Cytogenetic location: 6q21 Genomic coordinates (GRCh38): 6:107,028,199-107,051,586 (from NCBI)
MTRES1 protects cells from mitochondrial RNA loss during stress (Kotrys et al., 2019).
By sequencing clones obtained from a CD34 (142230)-positive human hematopoietic stem/progenitor cell cDNA library, Zhang et al. (2000) cloned MTRES1, which they termed HSPC230. The predicted protein contains 240 amino acids.
By database analysis and ectopic expression in 293 cells, Kotrys et al. (2019) identified human MTRES1 as a monomeric mitochondrial matrix RNA-binding protein. Edman degradation revealed that the N-terminal 84 amino acids of the MTRES1 preprotein were cleaved to form the mature protein.
Using RNA-sequencing analysis, Kotrys et al. (2019) showed that depletion of MTRES1 led to a reduction of 7S RNA originating from transcription of the noncoding regulatory region. In contrast, upregulation of MTRES1 prevented stress-induced reduction of mitochondrial transcription. The protective function of MTRES1 occurred at the mitochondrial transcriptional level, but not through changes in mitochondrial DNA levels, and depended on its RNA-binding ability. Immunoprecipitation analysis revealed that MTRES1 interacted with the mitochondrial transcription machinery constituents POLRMT (601778) and TFAM (600438).
The human mitochondrial ribosome (mitoribosome) and associated proteins regulate the synthesis of 13 essential subunits of the oxidative phosphorylation complexes. Desai et al. (2020) identified a human mitoribosome-associated quality control pathway that responded to interruptions during elongation, and they presented structures at 3.1- to 3.3-angstrom resolution of mitoribosomal large subunits trapped during ribosome rescue. The release factor homolog C12ORF65 (613541) and RNA-binding protein C6ORF203 ejected the nascent chain and peptidyl tRNA, respectively, from stalled ribosomes. Recruitment of mitoribosome biogenesis factors to quality control intermediates suggested additional roles for these factors during mitoribosome rescue. Desai et al. (2020) also reported related cryoelectron microscopy structures at 3.7- to 4.4-angstrom resolution of elongating mitoribosomes bound to tRNAs, nascent polypeptides, the guanosine triphosphatase elongation factors TUFM (602389) and GFM1 (606639), and the OXA1L translocase (601066).
By database analysis, Zhang et al. (2000) determined that the MTRES1 gene contains 4 exons.
Gross (2019) mapped the MTRES1 gene to chromosome 6q21 based on an alignment of the MTRES1 sequence (GenBank AF151064) with the genomic sequence (GRCh38).
Desai, N., Yang, H., Chandrasekaran, V., Kazi, R., Minczuk, M., Ramakrishnan, V. Elongational stalling activates mitoribosome-associated quality control. Science 370: 1105-1110, 2020. [PubMed: 33243891] [Full Text: https://doi.org/10.1126/science.abc7782]
Gross, M. B. Personal Communication. Baltimore, Md. 9/11/2019.
Kotrys, A. V., Cysewski, D., Czarnomska, S. D., Pietras, Z., Borowski, L. S., Dziembowski, A., Szczesny, R. J. Quantitative proteomics revealed C6orf203/MTRES1 as a factor preventing stress-induced transcription deficiency in human mitochondria. Nucleic Acids Res. 47: 7502-7517, 2019. [PubMed: 31226201] [Full Text: https://doi.org/10.1093/nar/gkz542]
Zhang, Q.-H., Ye, M., Wu, X.-Y., Ren, S.-X., Zhao, M., Zhao, C.-J., Fu, G., Shen, Y., Fan, H.-Y., Lu, G., Zhong, M., Xu, X.-R., and 9 others. Cloning and functional analysis of cDNAs with open reading frames for 300 previously undefined genes expressed in CD34+ hematopoietic stem/progenitor cells. Genome Res. 10: 1546-1560, 2000. [PubMed: 11042152] [Full Text: https://doi.org/10.1101/gr.140200]