Expression of GJB2 and GJB6 is reduced in a novel DFNB1 allele.

Wilch, Ellen; Zhu, Mei; Burkhart, Kirk B; Regier, Martha; Elfenbein, Jill L; Fisher, Rachel A; Friderici, Karen H · Am J Hum Genet · 2006

basic_science · Level V

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Abstract

In a large kindred of German descent, we found a novel allele that segregates with deafness when present in trans with the 35delG allele of GJB2. Qualitative polymerase chain reaction-based allele-specific expression assays showed that expression of both GJB2 and GJB6 from the novel allele is dramatically reduced. This is the first evidence of a deafness-associated regulatory mutation of GJB2 and of potential coregulation of GJB2 and GJB6.

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