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. Author manuscript; available in PMC: 2009 Feb 12.
Published in final edited form as: Laryngoscope. 2008 May;118(5):821–831. doi: 10.1097/MLG.0b013e318164d0aa

TABLE III.

Few of the Potential Targets for Gene Therapy

Gene Symbol Gene Product Function Location Mouse Model Syndromic Locus Nonsyndromic Locus References
MYO7A Myosin VIIa Intracellular motor 11q12.3 Shaker-1 USH1B DFNA11, B2 85, 86
USH1C Harmonin Scaffold protein 11p15.1 Deaf circler USH1C DFNB18 87
CDH23 Cadherin 23 Intercellular adhesion 10q21-q22 Waltzer USH1D DFNB12 88, 89
PCDH15 Protocadherin 15 Intercellular adhesion 10q21 Ames waltzer USH1F DFNB23 90, 91
SANS SANS Scaffold protein 17q24-q25 Jackson circler USH1G 92
MYO6 Myosin VI Intracellular motor 6q13 Snell’s waltzer DFNA22, B37 93, 94
KCNQ1 KCNQ1 Potassium channel 11p15.5 JLNS1 51
KCNE1 KCNE1 Potassium channel 21q22.1-q22.2 JLNS2 52
MYO15 Myosin 15 Intracellular motor 17p11.2 Shaker-2 DFNB3 95, 96

For complete list, see Hereditary Hearing Loss Homepage, http://webhost.ua.ac.be/hhh.