. Author manuscript; available in PMC: 2015 Jan 1.

Published in final edited form as: Int J Biochem Cell Biol. 2013 Nov 12;46:80–89. doi: 10.1016/j.biocel.2013.11.001

Table 1.

Usher genes and their associated disease.

Usher gene	Protein	Associated diseases	Mouse models	Zebrafish models	References
MY07A	Myosin VIIA	USH1 BDFNA11 DFNB2, non-syndromic RP	shaker1; Myo7A null	mariner	Sang et al. (2013); Ammar-Khodja et al. (2009); Weil et al. (1995); Ernest et al. (2000); Ben Rebeh et al. (2010); Lopes et al. (2013)
USH1C	Harmonin	USH1C DFNB18	dfcr; ush1c−/−; harmonin-PDZ2; Ush1c216AA	Ush1c^fh293	Johnson et al. (2003); Verpy et al. (2000); Lefevre et al. (2008); Grillet et al. (2009); Lentz et al. (2010); Tian et al. (2001); Seiler et al. (2004);
CDH23	Cadherin 23	USH1D DFNB12	Waltzer	sputnik	Bolz et al. (2001); Schultz et al. (2011); Sollner et al. (2004)
PCDH15	Protocadherin15	USH1F DFNB23	Ames Waltzer; noddy; pcdh15-CD1/CD2/CD3	arbiter	Ahmed et al. (2003); Ahmed et al. (2001); Seiler et al. (2004); Webb et al. (2011); Geng et al. (2013)
USH1G	SANS	USH1G DFNA20/26	Jackson shaker/Ush1g^js/js		Weil et al. (2003); Mustapha et al. (2006); Liu et al. (2007);
CIB2	Calcium integrin binding protein 2	USH1F DFNB48	Cib2tm1a(EUC0MM)^Wtsi		Riazuddin et al., 2012; MGI
USH2A	Usherin	USH2A non-syndromic RP (RP15)	Ush2a−/−	Ush2a^sa1881	Ebermann et al. (2009); Kremer et al. (2006); Liu et al. (2007); Xu et al. (2011); ZIRC
GPR98	Vlgr1/Gpr98	USH2C	VLGR1/del7TM; VLGR1 ko		Weston et al. (2004); McMillan and White (2004); Yagi et al. (2005)
DFNB31	Whirlin	USH2D DFNB31	Whirler; whirlinko		van Wijk et al. (2006); Mogensen et al. (2007); Mburu et al. (2006); Zou et al. (2011)
CLRN1	Clarin-1	USH3A	Clrn1(−/−)		Adato et al. (2002); Zallocchi et al. (2009)