. 2010 Sep 28;5(9):e12742. doi: 10.1371/journal.pone.0012742

Table 3. Top-ranked candidate genes.

Gene symbol	Gene description		Ear expression	Gene Functions for NSHL
WDR1	WD repeat domain 1	0.55	H. sapiens (ear)^a	regulation of hair
			M. musculus (inner ear)^a	cell actin dynamics^f
ALMS1	Alström syndrome 1	0.53	M. musculus (inner ear)^a	normal function of cilia [93]
CD151	CD151 molecule	0.52	possible human inner ear component [94]	inner ear ECM assembly [94]
	(Raph blood group)		M. musculus (inner ear)^a
CLRN1	clarin 1	0.52	M. musculus (inner ear)^a	inner ear development^f
			widely expressed in human^b	F actin organization^f
				protein trafficking^f
ABHD5	abhydrolase domain containing 5	0.52	M. musculus (inner ear)^a	TG accumulation^f
				lipid homeostasis^f
USH1G	Usher syndrome 1G	0.52	H. sapiens (inner ear)^b ^, ^c	cohesion of hair cell bundles^f
				(ankyrin and pdz domains)
ATP6V0A4	ATPase H transporting	0.51	H. sapiens (cochlea)^b [95]	cochlear pH homeostasis [96]
	lysosomal V0 subunit a4
PRCD	progressive rod-cone degeneration	0.50	no data	no evidence
KCNQ1	potassium voltage-gated channel	0.50	M. musculus (inner ear)^a	K cycling^f
	KQT-like subfamily member 1
NUMB	numb homolog (Drosophila)	0.50	H. sapiens (ear)^a	cell fate determination
			M. musculus (inner ear)^a	during development^f
ZAR1	zygote arrest 1	0.50	M. musculus (cochlea, stria vascularis)^g	no evidence
PTPLA	protein tyrosine phosphatase-like	0.50	H. sapiens (fetal cochlea)^d	signal transduction^f
	(proline instead of catalytic arginine)
	member A
FLII	flightless I homolog (Drosophila)	0.50	H. sapiens (fetal cochlea)^d	actin remodeling
PTPN11	protein tyrosine phosphatase	0.49	H. sapiens (ear)^a	signal transduction^f
	non-receptor type 11		M. musculus (inner ear)^a
TBL1X	transducin (beta)-like 1X-linked	0.49	H. sapiens (fetal cochlea)^d	signal transduction^f
			M. musculus (inner ear)	vescicular trafficking^f
				cytoskeleton assembly^f
KCNE1L	KCNE1-like	0.49	M. musculus (inner ear)^a	K cycling^f
TIMM8A	translocase of inner mitochondrial	0.49	no data	signal transduction^f
	membrane 8 homolog A (yeast)			protein transport^f
ROM1	retinal outer segment	0.49	H. sapiens (fetal cochlea)^d	cell adhesion^f
	membrane protein 1
CC2D2A	coiled-coil and C2	0.49	no data	Ca binding^f
	domain containing 2A			cilia formation^f
BARHL1	BarH-like homeobox 1	0.48	M.musculus (inner ear)^e	external sensory organ
				fate determination [97]

Gene expression information are taken from

NCBI Unigene [98],

UniProtKB [99],

HPRD database [100],

Morton Cochlear EST database [101], NCBI GEO [102],

the table of gene expression in the developing ear from the Institute of Hearing Research [103],

Bgee dataBase for Gene Expression Evolution [104] and literature. Gene function information have been inferred from

NCBI Gene [39] and literature.