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Protein Page:
plakophilin 2 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
plakophilin 2 May play a role in junctional plaques. Defects in PKP2 are the cause of familial arrhythmogenic right ventricular dysplasia type 9 (ARVD9); also known as arrhythmogenic right ventricular cardiomyopathy 9 (ARVC9). ARVD is an autosomal dominant disease characterized by partial degeneration of the myocardium of the right ventricle, electrical instability, and sudden death. It is clinically defined by electrocardiographic and angiographic criteria; pathologic findings, replacement of ventricular myocardium with fatty and fibrous elements, preferentially involve the right ventricular free wall. Belongs to the beta-catenin family. 2 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Motility/polarity/chemotaxis; Membrane protein, integral; Cell adhesion
Cellular Component: desmosome; adherens junction; integral to membrane; nucleolus; plasma membrane; intermediate filament; intercellular junction; cell junction; nucleus
Molecular Function: protein binding; protein kinase C binding; sodium channel regulator activity; intermediate filament binding; protein complex scaffold
Biological Process: negative regulation of cell proliferation; cell-cell adhesion; gap junction assembly; heart development; ventricular cardiac muscle morphogenesis; lipid homeostasis; intermediate filament bundle assembly; maintenance of organ identity; negative regulation of cell migration
Reference #:  Q99959 (UniProtKB)
Alt. Names/Synonyms: ARVD9; MGC177501; PKP2; plakophilin 2; Plakophilin-2
Gene Symbols: PKP2
Molecular weight: 97,415 Da
Basal Isoelectric point: 9.39  Predict pI for various phosphorylation states
Select Structure to View Below

plakophilin 2

Protein Structure Not Found.


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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


 SS 

SS: 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.


 MS 

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


       human

► Hide Isoforms
 
0 22 Y10-p APGAPAEyGyIRTVL
0 7 Y12-p GAPAEyGyIRTVLGQ
0 1 S29 LGQLDSSSLALPSEA
0 22 K37-ub LALPSEAkLkLAGSs
0 7 K39-ub LPSEAkLkLAGSsGr
0 1 S44-p kLkLAGSsGrGGQTV
0 6 R46-m1 kLAGSsGrGGQTVkS
0 5 K52-ub GrGGQTVkSLRIQEQ
0 2 T63-p IQEQVQQtLARKGRS
0 1 S71-p LARKGRSsVGNGNLH
0 10 T80-p GNGNLHRtssVPEyV
0 9 S81-p NGNLHRtssVPEyVy
1 120 S82-p GNLHRtssVPEyVyN
0 88 Y86-p RtssVPEyVyNLHLV
0 34 Y88-p ssVPEyVyNLHLVEN
0 17 S102-p NDFVGGRsPVPktyD
0 1 P105 VGGRsPVPktyDMLk
0 7 K106-ub GGRsPVPktyDMLkA
0 2 T107-p GRsPVPktyDMLkAG
0 30 Y108-p RsPVPktyDMLkAGT
0 11 K112-ub PktyDMLkAGTTAty
0 14 T118-p LkAGTTAtyEGRWGr
0 124 Y119-p kAGTTAtyEGRWGrG
0 3 R125-m1 tyEGRWGrGtAQyss
0 1 T127-p EGRWGrGtAQyssQk
0 32 Y130-p WGrGtAQyssQksVE
0 7 S131-p GrGtAQyssQksVEE
0 29 S132-p rGtAQyssQksVEER
0 20 K134-ub tAQyssQksVEERSL
0 59 S135-p AQyssQksVEERSLR
0 97 S151-p PLRRLEIsPDssPER
0 38 S154-p RLEIsPDssPERAHy
0 37 S155-p LEIsPDssPERAHyt
0 89 Y161-p ssPERAHytHsDyQy
0 29 T162-p sPERAHytHsDyQys
0 36 S164-p ERAHytHsDyQysQR
0 306 Y166-p AHytHsDyQysQRsQ
0 172 Y168-p ytHsDyQysQRsQAG
0 14 S169-p tHsDyQysQRsQAGH
0 1 S172-p DyQysQRsQAGHtLH
0 1 T177-p QRsQAGHtLHHQEsR
0 1 S183-p HtLHHQEsRRAALLV
0 7 V190 sRRAALLVPPRyARs
0 3 Y194-p ALLVPPRyARsEIVG
0 12 S197-p VPPRyARsEIVGVsR
0 2 S203-p RsEIVGVsRAGttSR
0 7 T207-p VGVsRAGttSRQRHF
0 1 T208-p GVsRAGttSRQRHFD
0 3 T216-p SRQRHFDtyHRQyQH
0 78 Y217-p RQRHFDtyHRQyQHG
0 6 Y221-p FDtyHRQyQHGsVSD
0 1 S225-p HRQyQHGsVSDTVFD
0 1 Y243-p ANPALLTyPRPGtSR
0 4 T248-p LTyPRPGtSRsMGNL
0 1 S249 TyPRPGtSRsMGNLL
0 49 S251-p PRPGtSRsMGNLLEk
0 2 N254 GtSRsMGNLLEkENy
0 9 K258-ub sMGNLLEkENyLtAG
0 71 Y261-p NLLEkENyLtAGLtV
0 16 T263-p LEkENyLtAGLtVGQ
0 2 T267-p NyLtAGLtVGQVRPL
0 1 A285 QPVTQNRASRsSWHQ
0 1 S288-p TQNRASRsSWHQssF
0 1 Q292 ASRsSWHQssFHsTR
0 2 S293-p SRsSWHQssFHsTRT
0 12 S294-p RsSWHQssFHsTRTL
0 4 S297-p WHQssFHsTRTLREA
0 2 T298 HQssFHsTRTLREAG
0 11 S329-p GQAAAGGsGNLLTER
0 8 K405-ub LKLLQLLkVQNEDVQ
0 3 K447-ub PRLLQVLkQTRDLET
0 1 T459-p LETKKQItDHtVNLR
0 1 T462-p KKQItDHtVNLRSRN
0 6 K514 WNLSSNDKLKNLMIT
0 1 K516 LSSNDKLKNLMITEA
0 1 S566 TGCLRNMSSAGADGR
0 20 Y597-p VRGTIADyQPDDKAT
0 2 Y616-p CILHNLSyQLEAELP
0 1 Y626-p EAELPEKySQNIyIQ
0 1 N629 LPEKySQNIyIQNRN
0 98 Y631-p EKySQNIyIQNRNIQ
0 6 K643-ub NIQTDNNkSIGCFGS
0 1 S644 IQTDNNkSIGCFGSR
0 1 S650 kSIGCFGSRSRKVKE
0 1 K656 GSRSRKVKEQyQDVP
0 62 Y659-p SRKVKEQyQDVPMPE
0 1 Y686-p HSIVIRMyLSLIAKs
0 1 S693-p yLSLIAKsVRNYTQE
0 1 K736-ub SGLQHTRkMLHVGDP
0 1 S744-p MLHVGDPsVKKTAIS
0 23 Y831-p AISAGDAyAsNKASK
0 1 S833-p SAGDAyAsNKASKAA
0 1 K864-ub YKKAQFKkTDFVNsR
0 2 S870-p KkTDFVNsRTAKAyH
0 4 Y876-p NsRTAKAyHsLKD__
0 1 S878-p RTAKAyHsLKD____
  plakophilin 2 iso2  
Y10 APGAPAEYGYIRTVL
Y12 GAPAEYGYIRTVLGQ
S29 LGQLDSSSLALPSEA
K37 LALPSEAKLKLAGSS
K39 LPSEAKLKLAGSSGR
S44 KLKLAGSSGRGGQTV
R46 KLAGSSGRGGQTVKS
K52 GRGGQTVKSLRIQEQ
T63 IQEQVQQTLARKGRS
S71 LARKGRSSVGNGNLH
T80 GNGNLHRTSSVPEYV
S81 NGNLHRTSSVPEYVY
S82 GNLHRTSSVPEYVYN
Y86 RTSSVPEYVYNLHLV
Y88 SSVPEYVYNLHLVEN
S102 NDFVGGRSPVPKTYD
P105 VGGRSPVPKTYDMLK
K106 GGRSPVPKTYDMLKA
T107 GRSPVPKTYDMLKAG
Y108 RSPVPKTYDMLKAGT
K112 PKTYDMLKAGTTATY
T118 LKAGTTATYEGRWGR
Y119 KAGTTATYEGRWGRG
R125 TYEGRWGRGTAQYSS
T127 EGRWGRGTAQYSSQK
Y130 WGRGTAQYSSQKSVE
S131 GRGTAQYSSQKSVEE
S132 RGTAQYSSQKSVEER
K134 TAQYSSQKSVEERSL
S135 AQYSSQKSVEERSLR
S151 PLRRLEISPDSSPER
S154 RLEISPDSSPERAHY
S155 LEISPDSSPERAHYT
Y161 SSPERAHYTHSDYQY
T162 SPERAHYTHSDYQYS
S164 ERAHYTHSDYQYSQR
Y166 AHYTHSDYQYSQRSQ
Y168 YTHSDYQYSQRSQAG
S169 THSDYQYSQRSQAGH
S172 DYQYSQRSQAGHTLH
T177 QRSQAGHTLHHQESR
S183 HTLHHQESRRAALLV
V190 SRRAALLVPPRYARS
Y194 ALLVPPRYARSEIVG
S197 VPPRYARSEIVGVSR
S203 RSEIVGVSRAGTTSR
T207 VGVSRAGTTSRQRHF
T208 GVSRAGTTSRQRHFD
T216 SRQRHFDTYHRQYQH
Y217 RQRHFDTYHRQYQHG
Y221 FDTYHRQYQHGSVSD
S225 HRQYQHGSVSDTVFD
Y243 ANPALLTYPRPGTSR
T248 LTYPRPGTSRSMGNL
S249 TYPRPGTSRSMGNLL
S251 PRPGTSRSMGNLLEK
N254 GTSRSMGNLLEKENY
K258 SMGNLLEKENYLTAG
Y261 NLLEKENYLTAGLTV
T263 LEKENYLTAGLTVGQ
T267 NYLTAGLTVGQVRPL
A285 QPVTQNRASRSSWHQ
S288 TQNRASRSSWHQSSF
Q292 ASRSSWHQSSFHSTR
S293 SRSSWHQSSFHSTRT
S294 RSSWHQSSFHSTRTL
S297 WHQSSFHSTRTLREA
T298 HQSSFHSTRTLREAG
S329 GQAAAGGSGNLLTER
K405 LKLLQLLKVQNEDVQ
K447 PRLLQVLKQTRDLET
T459 LETKKQITGLLWNLS
- gap
K470-ub WNLSSNDkLkNLMIT
K472-ub LSSNDkLkNLMITEA
S522 TGCLRNMSSAGADGR
Y553 VRGTIADYQPDDKAT
Y572 CILHNLSYQLEAELP
Y582 EAELPEKYSQNIYIQ
N585 LPEKYSQNIYIQNRN
Y587 EKYSQNIYIQNRNIQ
K599 NIQTDNNKSIGCFGS
S600 IQTDNNKSIGCFGSR
S606 KSIGCFGSRSRKVKE
K612 GSRSRKVKEQYQDVP
Y615 SRKVKEQYQDVPMPE
Y642 HSIVIRMYLSLIAKS
S649 YLSLIAKSVRNYTQE
K692 SGLQHTRKMLHVGDP
S700 MLHVGDPSVKKTAIS
Y787 AISAGDAYASNKASK
S789 SAGDAYASNKASKAA
K820 YKKAQFKKTDFVNSR
S826 KKTDFVNSRTAKAYH
Y832 NSRTAKAYHSLKD__
S834 RTAKAYHSLKD____
  mouse

 
C10 VPGSLAECGYIRTVL
Y12 GSLAECGYIRTVLGQ
S29-p LGHLDSSsLALPSEA
R37 LALPSEARLRLAGSS
R39 LPSEARLRLAGSSGR
S44 RLRLAGSSGRGDPAA
R46 RLAGSSGRGDPAARS
R52 GRGDPAARSQRIQEQ
T63 IQEQVQQTLARRGRS
S71 LARRGRSSAVSGNLH
T80-p VSGNLHRtssVPEyV
S81-p SGNLHRtssVPEyVY
S82-p GNLHRtssVPEyVYK
Y86-p RtssVPEyVYKLHVV
Y88 ssVPEyVYKLHVVEN
S102-p NDFVGRQsPVtRDYD
T105-p VGRQsPVtRDYDMLk
R106 GRQsPVtRDYDMLkA
D107 RQsPVtRDYDMLkAG
Y108 QsPVtRDYDMLkAGM
K112-ub tRDYDMLkAGMTATy
T118 LkAGMTATyGSRWGR
Y119-p kAGMTATyGSRWGRA
R125 TyGSRWGRAAAQYss
A127 GSRWGRAAAQYssQk
Y130 WGRAAAQYssQksVE
S131-p GRAAAQYssQksVEE
S132-p RAAAQYssQksVEER
K134-ub AAQYssQksVEERSW
S135-p AQYssQksVEERSWR
S151-p PLRRLEIsPDssPER
S154-p RLEIsPDssPERAHy
S155-p LEIsPDssPERAHyG
Y161-p ssPERAHyGHSEyQy
G162 sPERAHyGHSEyQyA
S164 ERAHyGHSEyQyAWR
Y166-p AHyGHSEyQyAWRSH
Y168-p yGHSEyQyAWRSHVV
A169 GHSEyQyAWRSHVVP
S172 EyQyAWRSHVVPGGR
- gap
- gap
T181-p VVPGGRLtLPRYARs
Y185 GRLtLPRYARsEILG
S188-p tLPRYARsEILGLRQ
R194 RsEILGLRQAGTARR
T198 LGLRQAGTARRPPGC
- gap
- gap
- gap
- gap
S207 RRPPGCGSFSDAVFD
H225 LKPTMPTHPPGtsHs
T229-p MPTHPPGtsHsAGsL
S230-p PTHPPGtsHsAGsLL
S232-p HPPGtsHsAGsLLEE
S235-p GtsHsAGsLLEETTV
E239 sAGsLLEETTVRVSQ
- gap
- gap
- gap
T256-p LQSTQSRtARSSWPr
S259 TQSRtARSSWPrssV
R263-m2 tARSSWPrssVRssL
S264-p ARSSWPrssVRssLR
S265-p RSSWPrssVRssLRE
S268-p WPrssVRssLREPGR
S269-p PrssVRssLREPGRM
S287-p AGQAAVGsGDAHGDR
K363-ub PKLLQLLkLQNEDVQ
K405 PRLLQVLKQTRDLET
T417 LETKKQITGLLWNLS
- gap
K428-ub WNLSSSDkLKHLMIT
K430 LSSSDkLKHLMITEA
S480-p TGCLRNMsSAGPDGR
Y511 VRGTIADYQPDDKAT
Y530 CILHNLSYQLEAELP
Y540 EAELPEKYSQsIyMQ
S543-p LPEKYSQsIyMQNRN
Y545-p EKYSQsIyMQNRNIQ
K557-ub NIQTNSNksIGCFGs
S558-p IQTNSNksIGCFGsR
S564-p ksIGCFGsRSRKLkE
K570-ub GsRSRKLkEQyQDLQ
Y573-p SRKLkEQyQDLQMPE
Y600 HSIVIRMYLSLIAKS
S607 YLSLIAKSTRNYTQE
K650 NGLQHTRKMLHVGDP
S658 MLHVGDPSVKKTAVS
Y745 TISIGEGYAPNKASK
P747 SIGEGYAPNKASKAA
K778 YKKAQFKKTDFVNsR
S784-p KKTDFVNsRTAKAYH
Y790 NsRTAKAYHSLKD__
S792 RTAKAYHSLKD____
  rat

 
C10 IPGSLGECGYIRTVL
Y12 GSLGECGYIRTVLGQ
S29 LGHLDSSSLALPSEA
R37 LALPSEARLRLAGSS
R39 LPSEARLRLAGSSGR
S44 RLRLAGSSGRGDPVA
R46 RLAGSSGRGDPVARS
R52 GRGDPVARSQRIQEQ
T63 IQEQVQQTLARRGRS
S71 LARRGRSSVVGGNLH
T80 VGGNLHRTSsVPEYV
S81 GGNLHRTSsVPEYVY
S82-p GNLHRTSsVPEYVYK
Y86 RTSsVPEYVYKLHLV
Y88 SsVPEYVYKLHLVEN
S102 NDFVGRQSPAARDYD
A105 VGRQSPAARDYDMLK
R106 GRQSPAARDYDMLKA
D107 RQSPAARDYDMLKAG
Y108 QSPAARDYDMLKAGV
K112 ARDYDMLKAGVTATy
T118 LKAGVTATyGSRWGR
Y119-p KAGVTATyGSRWGRG
R125 TyGSRWGRGTAQYSS
T127 GSRWGRGTAQYSSQK
Y130 WGRGTAQYSSQKSVE
S131 GRGTAQYSSQKSVEE
S132 RGTAQYSSQKSVEER
K134 TAQYSSQKSVEERSW
S135 AQYSSQKSVEERSWR
S151-p PLRRLEIsPDSsPER
S154 RLEIsPDSsPERAHY
S155-p LEIsPDSsPERAHYG
Y161 SsPERAHYGHSEYQY
G162 sPERAHYGHSEYQYA
S164 ERAHYGHSEYQYAWR
Y166 AHYGHSEYQYAWRSH
Y168 YGHSEYQYAWRSHVV
A169 GHSEYQYAWRSHVVP
S172 EYQYAWRSHVVPGGR
- gap
- gap
T181 VVPGGRLTLPRYARS
Y185 GRLTLPRYARSEILG
S188 TLPRYARSEILGLRQ
R194 RSEILGLRQTGTARR
T198 LGLRQTGTARRAPGC
- gap
- gap
- gap
- gap
S207 RRAPGCGSVSDAVFD
H225 LNPTIPTHPPGTSHS
T229 IPTHPPGTSHSAGSL
S230 PTHPPGTSHSAGSLL
S232 HPPGTSHSAGSLLEE
S235 GTSHSAGSLLEETTV
E239 SAGSLLEETTVRVSQ
- gap
- gap
- gap
A256 FQGLQSRAGRSSWPR
S259 LQSRAGRSSWPRSSV
R263 AGRSSWPRSSVHSTL
S264 GRSSWPRSSVHSTLR
S265 RSSWPRSSVHSTLRE
S268 WPRSSVHSTLRESGR
T269 PRSSVHSTLRESGRM
S287 AGQAAVGSGDAHRDR
K363 PKLLQLLKVQNEDVQ
K405 PRLLQVLKQTRDLET
T417 LETKKQITGLLWNLS
- gap
K428 WNLSSSDKLKHLMIT
K430 LSSSDKLKHLMITEA
S480 TGCLRNMSSAGPDGR
Y511 VRGTIADYQPDDKAT
Y530 CILHNLSYQLEAELP
Y540 EAELPEKYSQSIYMQ
S543 LPEKYSQSIYMQNRN
Y545 EKYSQSIYMQNRNIQ
K557 NIQTNSNKSIGCFGS
S558 IQTNSNKSIGCFGSR
S564 KSIGCFGSRSRKVKE
K570 GSRSRKVKEQYQDTP
Y573 SRKVKEQYQDTPMPE
Y600 HSIVIRMYLSLIAKS
S607 YLSLIAKSSRNYTQE
K650 NGLQHTRKMLHVGDP
S658 MLHVGDPSVKKTAVS
Y745 TISIGEGYAPNKASK
P747 SIGEGYAPNKASKAA
K778 YKKAQFKKTDFVNSR
S784 KKTDFVNSRTAKASH
S790 NSRTAKASHSLKD__
S792 RTAKASHSLKD____
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