The role of mitochondrial tRNAPhe C628T variant in deafness expression

Mitochondrial DNA. 2015 Feb;26(1):2-6. doi: 10.3109/19401736.2013.823192. Epub 2013 Sep 11.

Abstract

Mutations in mitochondrial genome are one of the most important causes of hearing loss, of these, mitochondrial tRNA (mt-tRNA) genes are the hot spots for mutations associated with deafness. Most recently, a novel mt-tRNA(Phe) C628T variant has been reported to be associated with non-syndromic and sensorineural hearing loss. To test this association, we characterized the C628T variant using a phylogenetic approach; in addition, we employed the bioinformatics tool to predict the thermodynamic change of the mt-tRNA(Phe) gene with and without this variant. Intriguingly, the C628T variant was not evolutionary conserved and had little effect on mt-tRNA(Phe) folding. Moreover, through the application of the pathogenicity scoring system, we classified the C628T variant as a "neutral polymorphism", suggesting that this variant currently lacked sufficient evident to support as a "pathogenic" mutation.

Keywords: C628T variant; deafness; mitochondrial tRNAPhe; pathogenic.

MeSH terms

  • Alleles
  • Base Sequence
  • Computational Biology
  • Databases, Nucleic Acid
  • Deafness / diagnosis
  • Deafness / genetics*
  • Evolution, Molecular
  • Gene Expression*
  • Genes, Mitochondrial
  • Genetic Predisposition to Disease
  • Genetic Variation*
  • Genome, Mitochondrial
  • Humans
  • Mutation
  • Nucleic Acid Conformation
  • Phylogeny
  • RNA / chemistry
  • RNA / genetics*
  • RNA, Mitochondrial
  • RNA, Transfer, Phe / chemistry
  • RNA, Transfer, Phe / genetics*
  • Thermodynamics

Substances

  • RNA, Mitochondrial
  • RNA, Transfer, Phe
  • RNA