Genetic basis of Y-linked hearing impairment.

Wang, Qiuju; Xue, Yali; Zhang, Yujun; Long, Quan; Yang, Fengtang; Turner, Daniel J; Fitzgerald, Tomas; Ng, Bee Ling et al. · Am J Hum Genet · 2013

basic_science · Level V

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Abstract

A single Mendelian trait has been mapped to the human Y chromosome: Y-linked hearing impairment. The molecular basis of this disorder is unknown. Here, we report the detailed characterization of the DFNY1 Y chromosome and its comparison with a closely related Y chromosome from an unaffected branch of the family. The DFNY1 chromosome carries a complex rearrangement, including duplication of several noncontiguous segments of the Y chromosome and insertion of ∼160 kb of DNA from chromosome 1, in the pericentric region of Yp. This segment of chromosome 1 is derived entirely from within a known hearing impairment locus, DFNA49. We suggest that a third copy of one or more genes from the shared segment of chromosome 1 might be responsible for the hearing-loss phenotype.

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