Clue to a New Deafness Gene: A Large Chinese Nonsyndromic Hearing Loss Family Linked to DFNA4 |
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| Authors: | Liang Zong Chunye Lu Yali Zhao Qian Li Dongyi Han Weiyan Yang Yan Shen Qingyin Zheng Qiuju Wang |
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| Affiliation: | Liang Zong (Department of Otolaryngology, Head and Neck Surgery, Institute of Otolaryngology, Chinese PLA General Hospital,28 Fuxing Road, Beijing 100853, China); Chunye Lu (Chinese National Human Genome Centre, Beijing 100176, China); Yali Zhao (Department of Otolaryngology, Head and Neck Surgery, Institute of Otolaryngology, Chinese PLA General Hospital,28 Fuxing Road, Beijing 100853, China); Qian Li (Department of Otolaryngology, Head and Neck Surgery, Institute of Otolaryngology, Chinese PLA General Hospital,28 Fuxing Road, Beijing 100853, China); Dongyi Han (Department of Otolaryngology, Head and Neck Surgery, Institute of Otolaryngology, Chinese PLA General Hospital,28 Fuxing Road, Beijing 100853, China); Weiyan Yang (Department of Otolaryngology, Head and Neck Surgery, Institute of Otolaryngology, Chinese PLA General Hospital,28 Fuxing Road, Beijing 100853, China); Yan Shen (Chinese National Human Genome Centre, Beijing 100176, China ;Department of Biochemistry and Molecular Biology, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100853, China); Qingyin Zheng (Department of Otolaryngology-HNS, Case Western Reserve University,Cleveland, OH 44106, USA); Qiuju Wang (Department of Otolaryngology, Head and Neck Surgery, Institute of Otolaryngology, Chinese PLA General Hospital,28 Fuxing Road, Beijing 100853, China ;Chinese National Human Genome Centre, Beijing 100176, China); |
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| Abstract: | ![]()
Hereditary hearing loss is one of the most common neurosensory defects in humans.Approximately 70％ of cases are nonsyndromic and could be inherited in autosomal dominant,autosomal recessive,mitochondrial,X-linked,and Y-linked manners (Wang et al.,2004;Alford,2011).The autosomal dominant type,comprising 15％-20％ of nonsyndromic hearing loss,is monogenic and genetically heterogeneous.Since the first dominant deafness locus (DFNA1) was identified in 1992,a total of 64 DFNA loci have been mapped (DFNA1-DFNA64),and 27 corresponding genes have been identified (http://hereditaryhearingloss.org).Previous studies have revealed that one deafness locus can be linked to more than one gene (Bayazit and Yilmaz,2006),and the question "one locus,how many genes?" was first raised about a decade ago (Van-Hauwe et al.,1999).So far,several loci,including DFNA2 and DFNA3,have been shown to be related to one or more genes,showing high genetic heterogeneity in hereditary hearing loss (Grifa et al.,1999;Goldstein and Lalwani,2002;Yan et al.,2011). |
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