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Protein Page:
CDH23 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitylation
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
CDH23 Cadherins are calcium-dependent cell adhesion proteins. They preferentially interact with themselves in a homophilic manner in connecting cells. CDH23 is required for establishing and/or maintaining the proper organization of the stereocilia bundle of hair cells in the cochlea and the vestibule during late embryonic/early postnatal development. It is part of the functional network formed by USH1C, USH1G, CDH23 and MYO7A that mediates mechanotransduction in cochlear hair cells. Required for normal hearing. Defects in CDH23 are the cause of Usher syndrome type 1D (USH1D). USH is a genetically heterogeneous condition characterized by the association of retinitis pigmentosa and sensorineural deafness. Age at onset and differences in auditory and vestibular function distinguish Usher syndrome type 1 (USH1), Usher syndrome type 2 (USH2) and Usher syndrome type 3 (USH3). USH1 is characterized by profound congenital sensorineural deafness, absent vestibular function and prepubertal onset of progressive retinitis pigmentosa leading to blindness. Defects in CDH23 are a cause of Usher syndrome type 1D/F (USH1DF). USH1DF patients are heterozygous for mutations in CDH23 and PCDH15, indicating a digenic inheritance pattern. Defects in CDH23 are the cause of deafness autosomal recessive type 12 (DFNB12). DFNB12 is a form of sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information. 8 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Cell adhesion; Motility/polarity/chemotaxis; Membrane protein, integral
Chromosomal Location of Human Ortholog: 10q22.1
Cellular Component: integral to membrane; membrane; plasma membrane; stereocilium
Molecular Function: calcium ion binding; protein binding
Biological Process: calcium ion transport; calcium-dependent cell-cell adhesion; cytosolic calcium ion homeostasis; equilibrioception; homophilic cell adhesion; inner ear receptor stereocilium organization and biogenesis; locomotory behavior; photoreceptor cell maintenance; response to stimulus; sensory perception of light stimulus; sensory perception of sound; visual perception
Disease: Deafness, Autosomal Recessive 12; Usher Syndrome, Type Id
Reference #:  Q9H251 (UniProtKB)
Alt. Names/Synonyms: CAD23; Cadherin-23; cadherin-like 23; cadherin-related 23; cadherin-related family member 23; CDH23; CDHR23; DKFZp434P2350; FLJ00233; FLJ36499; KIAA1774; KIAA1812; MGC102761; Otocadherin; USH1D
Gene Symbols: CDH23
Molecular weight: 369,494 Da
Basal Isoelectric point: 4.5  Predict pI for various phosphorylation states
Select Structure to View Below

CDH23

Protein Structure Not Found.
Download PyMol Script
Download ChimeraX Script


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 LTP 

LTP: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 HTP 

HTP: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

► Hide Isoforms
 
0 1 Y201‑p DYETTQAyQLTVNAt
0 1 T208‑p yQLTVNAtDQDKtRP
0 1 T213‑p NAtDQDKtRPLSTLA
0 1 S461 NDNRPIFSQPLYNIS
0 1 - gap
0 1 Y523‑p MLIARLDyELIQRFT
0 1 Y704‑p DLDRSREyGQESIIy
0 1 Y711‑p yGQESIIySLEGSTQ
0 1 S943‑p MDFLINSsSGVVVTT
0 1 T951‑p SGVVVTTtELDRERI
0 1 T1226‑p RETAGIGtSVIVVQA
0 1 Y1246‑p GDGGLVNyRILSGAE
0 1 K1442 GQRVATVKAWDPDAG
0 1 Y1672‑p GPNGTVTyAIVAGNI
0 1 S2155 DRGTVPLSGTAIVTI
0 1 T2161 LSGTAIVTILIDDIN
0 1 T2220‑p GIVAKDDtDRLVPNQ
0 1 T2237‑p AFAVNINtGsVMVKS
0 1 S2239‑p AVNINtGsVMVKSPM
0 2 S2259‑p ATYEVTLsVIDNASD
0 3 S2271‑p ASDLPERsVSVPNAK
0 1 T2280‑p SVPNAKLtVNVLDVN
0 1 T2290‑p VLDVNDNtPQFKPFG
0 1 S2562‑p AFHVDMDsGLVTTQR
0 1 Y2633‑p IPLRSNVyEVyATDK
0 1 Y2636‑p RSNVyEVyATDKDEG
0 1 Y2650‑p GLNGAVRySFLKTAG
0 2 S2729‑p FVRPPKGsPQyQLLt
0 1 Y2732‑p PPKGsPQyQLLtVPE
0 1 T2736‑p sPQyQLLtVPEHsPR
0 1 S2741‑p LLtVPEHsPRGTLVG
0 1 S2807‑p SFIVKASsNRsWtPP
0 1 S2810‑p VKASsNRsWtPPRGP
0 1 T2812‑p ASsNRsWtPPRGPSP
0 1 T2943‑p DLAGHNDtAIIGIyI
0 1 Y2949‑p DtAIIGIyILRDDQR
0 1 Y3089‑p LFVLMNWyyRTVHKR
0 1 Y3090‑p FVLMNWyyRTVHKRK
0 1 K3181‑ub GREPAAVkPDDDRYL
0 12 Y3195‑p LRAAIQEyDNIAKLG
  CDH23 iso5  
Y201 DYETTQAYQLTVNAT
T208 YQLTVNATDQDKTRP
T213 NATDQDKTRPLSTLA
S461 NDNRPIFSQPLYNIS
T489‑p VLVSPRFtAGPLSSP
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
  mouse

 
Y201 DYEVTQAYQLTVNAT
T208 YQLTVNATDQDKTRP
T213 NATDQDKTRPLSTLA
S461‑p NDNRPIFsQPLYNVS
- gap
Y523 MLIARLDYELIQRFT
Y704 DLDRSREYGQESIIY
Y711 YGQESIIYSLEGSSQ
T943 MDFVINSTSGVVTTT
A951 SGVVTTTAELDRERI
T1226 RETAGIGTSVIVVRA
Y1246 GDGGLVNYRILSGAE
K1442 GQRVATVKARDPDAG
Y1672 GPNGTVTYAIVAGNI
S2155‑p DRGTVPLsGTAIVtI
T2161‑p LsGTAIVtILIDDIN
T2220 SIVAKDDTDRLVPDQ
T2237 AFAVNINTGSVMVKS
S2239 AVNINTGSVMVKSPL
S2259 ATYEVTLSVIDNASD
S2271 ASDLPEHSVSVPNAK
T2280 SVPNAKLTVNILDVN
T2290 ILDVNDNTPQFKPFG
S2562 AFSVDMDSGLVTTQR
Y2633 IPLRSNVYEVYATDN
Y2636 RSNVYEVYATDNDEG
Y2650 GLNGAVRYSFLKTTG
S2729 FVRPPKGSPQYQLLT
Y2732 PPKGSPQYQLLTVPE
T2736 SPQYQLLTVPEHSPR
S2741 LLTVPEHSPRGTLVG
S2807 SFIVKASSNRSWTPP
S2810 VKASSNRSWTPPRGP
T2812 ASSNRSWTPPRGPSP
T2943‑p DLAGHNDtAIIGIyI
Y2949‑p DtAIIGIyILRDDQR
Y3089 LFVLMNWYYRTIHKR
Y3090 FVLMNWYYRTIHKRK
K3181 GREPAAVKPDDDRYL
Y3195 LRAAIQEYDNIAKLG
  rat

 
Y201 DYEVTQAYQLTVNAT
T208 YQLTVNATDQDKTRP
T213 NATDQDKTRPLSTLA
S459 NDNRPIFSQPLYNVS
- gap
Y521 MLIARLDYELIQRFT
Y702 DLDRSREYGQESIIY
Y709 YGQESIIYSLEGSSQ
T941 MDFVINSTSGVVTTT
A949 SGVVTTTAELDRERI
T1224 RETAGIGTSVIVVRA
Y1244 GDGGLVNYRILSGAE
K1440‑ac GQRVATVkARDPDAG
Y1670 GPNGTVTYAIVAGNI
S2153 DRGTVPLSGTATVTI
T2159 LSGTATVTILIDDIN
T2218 SIVAKDDTDRLVPDQ
T2235 AFAVNINTGSVIVKS
S2237 AVNINTGSVIVKSPL
S2257 ATYEVTLSVIDNASD
S2269 ASDLPERSVSVPNAK
T2278 SVPNAKLTVNILDVN
T2288 ILDVNDNTPQFKPFG
S2560 AFSVDMDSGLVTTQR
Y2631 IPLRSNVYEVYATDK
Y2634 RSNVYEVYATDKDEG
Y2648 GLNGAVRYSFLKSTG
S2727 FVRPPKGSPQYQLLT
Y2730 PPKGSPQYQLLTVPE
T2734 SPQYQLLTVPEHSPR
S2739 LLTVPEHSPRGTLVG
S2805 SFIVKASSNRSWTPP
S2808 VKASSNRSWTPPRGP
T2810 ASSNRSWTPPRGPSP
T2941 DLAGHNDTAIIGIYI
Y2947 DTAIIGIYILRDDQR
Y3087 LFVLMNWYYRTIHKR
Y3088 FVLMNWYYRTIHKRK
K3179 GREPAAVKPEDDRYL
Y3193 LRAAIQEYDNIAKLG
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