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188 related items for PubMed ID: 33045734

  • 1. Asymmetrical effects of deafness-associated mitochondrial DNA 7516delA mutation on the processing of RNAs in the H-strand and L-strand polycistronic transcripts.
    Xiao Y, Wang M, He Q, Xu L, Zhang Q, Meng F, Jia Z, Zhang F, Wang H, Guan MX.
    Nucleic Acids Res; 2020 Nov 04; 48(19):11113-11129. PubMed ID: 33045734
    [Abstract] [Full Text] [Related]

  • 2. Hypertension-associated mitochondrial DNA 4401A>G mutation caused the aberrant processing of tRNAMet, all 8 tRNAs and ND6 mRNA in the light-strand transcript.
    Zhao X, Cui L, Xiao Y, Mao Q, Aishanjiang M, Kong W, Liu Y, Chen H, Hong F, Jia Z, Wang M, Jiang P, Guan MX.
    Nucleic Acids Res; 2019 Nov 04; 47(19):10340-10356. PubMed ID: 31504769
    [Abstract] [Full Text] [Related]

  • 3. Biochemical characterization of the mitochondrial tRNASer(UCN) T7511C mutation associated with nonsyndromic deafness.
    Li X, Fischel-Ghodsian N, Schwartz F, Yan Q, Friedman RA, Guan MX.
    Nucleic Acids Res; 2004 Nov 04; 32(3):867-77. PubMed ID: 14960712
    [Abstract] [Full Text] [Related]

  • 4. A deafness-associated tRNAAsp mutation alters the m1G37 modification, aminoacylation and stability of tRNAAsp and mitochondrial function.
    Wang M, Peng Y, Zheng J, Zheng B, Jin X, Liu H, Wang Y, Tang X, Huang T, Jiang P, Guan MX.
    Nucleic Acids Res; 2016 Dec 15; 44(22):10974-10985. PubMed ID: 27536005
    [Abstract] [Full Text] [Related]

  • 5. A deafness-associated mitochondrial DNA mutation altered the tRNASer(UCN) metabolism and mitochondrial function.
    Xue L, Chen Y, Tang X, Yao J, Huang H, Wang M, Ye S, Wang M, Guan MX.
    Mitochondrion; 2019 May 15; 46():370-379. PubMed ID: 30336267
    [Abstract] [Full Text] [Related]

  • 6. Contribution of a mitochondrial tyrosyl-tRNA synthetase mutation to the phenotypic expression of the deafness-associated tRNASer(UCN) 7511A>G mutation.
    Fan W, Zheng J, Kong W, Cui L, Aishanjiang M, Yi Q, Wang M, Cang X, Tang X, Chen Y, Mo JQ, Sondheimer N, Ge W, Guan MX.
    J Biol Chem; 2019 Dec 13; 294(50):19292-19305. PubMed ID: 31685661
    [Abstract] [Full Text] [Related]

  • 7. Overexpression of mitochondrial histidyl-tRNA synthetase restores mitochondrial dysfunction caused by a deafness-associated tRNAHis mutation.
    Gong S, Wang X, Meng F, Cui L, Yi Q, Zhao Q, Cang X, Cai Z, Mo JQ, Liang Y, Guan MX.
    J Biol Chem; 2020 Jan 24; 295(4):940-954. PubMed ID: 31819004
    [Abstract] [Full Text] [Related]

  • 8. The deafness-associated mitochondrial DNA mutation at position 7445, which affects tRNASer(UCN) precursor processing, has long-range effects on NADH dehydrogenase subunit ND6 gene expression.
    Guan MX, Enriquez JA, Fischel-Ghodsian N, Puranam RS, Lin CP, Maw MA, Attardi G.
    Mol Cell Biol; 1998 Oct 24; 18(10):5868-79. PubMed ID: 9742104
    [Abstract] [Full Text] [Related]

  • 9. A new mechanism for mtDNA pathogenesis: impairment of post-transcriptional maturation leads to severe depletion of mitochondrial tRNASer(UCN) caused by T7512C and G7497A point mutations.
    Möllers M, Maniura-Weber K, Kiseljakovic E, Bust M, Hayrapetyan A, Jaksch M, Helm M, Wiesner RJ, von Kleist-Retzow JC.
    Nucleic Acids Res; 2005 Oct 24; 33(17):5647-58. PubMed ID: 16199753
    [Abstract] [Full Text] [Related]

  • 10. The 7472insC mitochondrial DNA mutation impairs the synthesis and extent of aminoacylation of tRNASer(UCN) but not its structure or rate of turnover.
    Toompuu M, Yasukawa T, Suzuki T, Hakkinen T, Spelbrink JN, Watanabe K, Jacobs HT.
    J Biol Chem; 2002 Jun 21; 277(25):22240-50. PubMed ID: 11919191
    [Abstract] [Full Text] [Related]

  • 11. Molecular phenotype of the np 7472 deafness-associated mitochondrial mutation in osteosarcoma cell cybrids.
    Toompuu M, Tiranti V, Zeviani M, Jacobs HT.
    Hum Mol Genet; 1999 Nov 21; 8(12):2275-83. PubMed ID: 10545608
    [Abstract] [Full Text] [Related]

  • 12. A deafness-associated tRNA mutation caused pleiotropic effects on the m1G37 modification, processing, stability and aminoacylation of tRNAIle and mitochondrial translation.
    Meng F, Zhou M, Xiao Y, Mao X, Zheng J, Lin J, Lin T, Ye Z, Cang X, Fu Y, Wang M, Guan MX.
    Nucleic Acids Res; 2021 Jan 25; 49(2):1075-1093. PubMed ID: 33398350
    [Abstract] [Full Text] [Related]

  • 13. In vitro 3'-end endonucleolytic processing defect in a human mitochondrial tRNA(Ser(UCN)) precursor with the U7445C substitution, which causes non-syndromic deafness.
    Levinger L, Jacobs O, James M.
    Nucleic Acids Res; 2001 Nov 01; 29(21):4334-40. PubMed ID: 11691920
    [Abstract] [Full Text] [Related]

  • 14. Mechanistic insights into mitochondrial tRNAAla 3'-end metabolism deficiency.
    Ji Y, Nie Z, Meng F, Hu C, Chen H, Jin L, Chen M, Zhang M, Zhang J, Liang M, Wang M, Guan MX.
    J Biol Chem; 2021 Jul 01; 297(1):100816. PubMed ID: 34023389
    [Abstract] [Full Text] [Related]

  • 15. A deafness-associated tRNAHis mutation alters the mitochondrial function, ROS production and membrane potential.
    Gong S, Peng Y, Jiang P, Wang M, Fan M, Wang X, Zhou H, Li H, Yan Q, Huang T, Guan MX.
    Nucleic Acids Res; 2014 Jul 01; 42(12):8039-48. PubMed ID: 24920829
    [Abstract] [Full Text] [Related]

  • 16. A deafness-associated mitochondrial DNA mutation caused pleiotropic effects on DNA replication and tRNA metabolism.
    Meng F, Jia Z, Zheng J, Ji Y, Wang J, Xiao Y, Fu Y, Wang M, Ling F, Guan MX.
    Nucleic Acids Res; 2022 Sep 09; 50(16):9453-9469. PubMed ID: 36039763
    [Abstract] [Full Text] [Related]

  • 17. Biochemical characterization of the deafness-associated mitochondrial tRNASer(UCN) A7445G mutation in osteosarcoma cell cybrids.
    Li X, Zhang LS, Fischel-Ghodsian N, Guan MX.
    Biochem Biophys Res Commun; 2005 Mar 11; 328(2):491-8. PubMed ID: 15694374
    [Abstract] [Full Text] [Related]

  • 18. Deafness-associated tRNAPhe mutation impaired mitochondrial and cellular integrity.
    Chen X, Meng F, Chen C, Li S, Chou Z, Xu B, Mo JQ, Guo Y, Guan MX.
    J Biol Chem; 2024 May 11; 300(5):107235. PubMed ID: 38552739
    [Abstract] [Full Text] [Related]

  • 19. Molecular phenotype of a human lymphoblastoid cell-line homoplasmic for the np 7445 deafness-associated mitochondrial mutation.
    Reid FM, Rovio A, Holt IJ, Jacobs HT.
    Hum Mol Genet; 1997 Mar 11; 6(3):443-9. PubMed ID: 9147648
    [Abstract] [Full Text] [Related]

  • 20. Biochemical Evidence for a Nuclear Modifier Allele (A10S) in TRMU (Methylaminomethyl-2-thiouridylate-methyltransferase) Related to Mitochondrial tRNA Modification in the Phenotypic Manifestation of Deafness-associated 12S rRNA Mutation.
    Meng F, Cang X, Peng Y, Li R, Zhang Z, Li F, Fan Q, Guan AS, Fischel-Ghosian N, Zhao X, Guan MX.
    J Biol Chem; 2017 Feb 17; 292(7):2881-2892. PubMed ID: 28049726
    [Abstract] [Full Text] [Related]

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