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Article

Auditory Pathology in a Transgenic mtTFB1 Mouse Model of Mitochondrial Deafness.

The American journal of pathology - 1 Dec 2015

McKay Sharen E, Yan Wayne, Nouws Jessica, Thormann Maximilian J, Raimundo Nuno, Khan Abdul, Santos-Sacchi Joseph, Song Lei, Shadel Gerald S

Abstract excerpt

The A1555G mutation in the 12S rRNA gene of human mitochondrial DNA causes maternally inherited, nonsyndromic deafness, an extreme case of tissue-specific mitochondrial pathology. A transgenic mouse strain that robustly overexpresses the mitochondrial 12S ribosomal RNA methyltransferase TFB1M (Tg-mtTFB1 mice) exhibits progressive hearing loss that we proposed models aspects of A1555G-related pathology in humans....

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Identifiers

PMID: 26552864
DOI: 10.1016/j.ajpath.2015.08.014
PMCID: PMC5801480

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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