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. Author manuscript; available in PMC: 2017 Jan 1.
Published in final edited form as: Wiley Interdiscip Rev Dev Biol. 2015 Aug 11;5(1):85–101. doi: 10.1002/wdev.202

Table 1.

Link type Composition Appearance
in mouse
Associated
proteins
/regulators
Associated
human
deafness loci
Permanent Tip CDH23
PCDH15
E17.5 myosin VIIa,
SANS, harmonin
TMHS/LHFPL5
USH1*
Horizontal top
connectors
stereocilin P9 None reported DFNB16
Transient Tansient
lateral links /
Shaft connectors
CDH23
PCDH15 /
PTPRQ?
E17.5 myosin VIIa,
SANS, harmonin
USH1*
Kinociliary CDH23
PCDH15-CD2
E17.5 myosin VIIa,
SANS, harmonin
USH1*
Ankle GPR98
VLGR1
usherin
P0 PDZD7, whirlin USH2*
*

This link type is also associated with additional forms of nonsydromic hearing loss. See http://hereditaryhearingloss.org for a comprehensive list.