Mitochondrial ribosomal RNA mutation associated with both antibiotic-induced and non-syndromic deafness

Toni R. Prezant, John V. Agapian, M. Charlotte Bohlman, Xiangdong Bu, Sitki Öztas, Wei Qin Qiu, Kathleen S. Arnos, Gino A. Cortopassi, Lutfi Jaber, Jerome I. Rotter, Mordechai Shohat, Nathan Fischel-Ghodsian*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

1055 Scopus citations

Abstract

Maternally transmitted non-syndromic deafness was described recently both in pedigrees with susceptibility to aminoglycoside ototoxicity and in a large Arab-Israeli pedigree. Because of the known action of aminoglycosides on bacterial ribosomes, we analysed the sequence of the mitochondrial rRNA genes of three unrelated patients with familial aminoglycoside-induced deafness. We also sequenced the complete mitochondrial genome of the Arab-Israeli pedigree. All four families shared a nucleotide 1555 A to G substitution in the 12S rRNA gene, a site implicated in aminoglycoside activity. Our study offers the first description of a mitochondrial rRNA mutation leading to disease, the first cases of non-syndromic deafness caused by a mitochondrial DNA mutation and the first molecular genetic study of antibiotic-induced ototoxicity.

Original languageEnglish
Pages (from-to)289-294
Number of pages6
JournalNature Genetics
Volume4
Issue number3
DOIs
StatePublished - Jul 1993
Externally publishedYes

Funding

FundersFunder number
National Institute on Deafness and Other Communication DisordersR01DC001402

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