NPHP3_HUMAN - dbPTM
NPHP3_HUMAN - PTM Information in dbPTM
Basic Information of Protein
UniProt ID NPHP3_HUMAN
UniProt AC Q7Z494
Protein Name Nephrocystin-3
Gene Name NPHP3
Organism Homo sapiens (Human).
Sequence Length 1330
Subcellular Localization Cell projection, cilium . Localization to cilium is mediated via interaction with UNC119 and UNC119B, which bind to the myristoyl moiety of the N-terminus.
Protein Description Required for normal ciliary development and function. Inhibits disheveled-1-induced canonical Wnt-signaling activity and may also play a role in the control of non-canonical Wnt signaling which regulates planar cell polarity. Probably acts as a molecular switch between different Wnt signaling pathways. Required for proper convergent extension cell movements..
Protein Sequence MGTASSLVSPAGGEVIEDTYGAGGGEACEIPVEVKPKARLLRNSFRRGAGAAAGAGPGSLPRGVGAGGLLGASFKSTGSSVPELEYAAAEYERLRKEYEIFRVSKNQELLSMGRREAKLDTENKRLRAELQALQKTYQKILREKESALEAKYQAMERAATFEHDRDKVKRQFKIFRETKENEIQDLLRAKRELESKLQRLQAQGIQVFDPGESDSDDNCTDVTAAGTQCEYWTGGALGSEPSIGSMIQLQQSFRGPEFAHSSIDVEGPFANVNRDDWDIAVASLLQVTPLFSHSLWSNTVRCYLIYTDETQPEMDLFLKDYSPKLKRMCETMGYFFHAVYFPIDVENQYLTVRKWEIEKSSLVILFIHLTLPSLLLEDCEEAFLKNPEGKPRLIFHRLEDGKVSSDSVQQLIDQVSNLNKTSKAKIIDHSGDPAEGVYKTYICVEKIIKQDILGFENTDLETKDLGSEDSIPEEDDFGDVLWDIHDEQEQMETFQQASNSAHELGFEKYYQRLNDLVAAPAPIPPLLVSGGPGSGKSLLLSKWIQLQQKNSPNTLILSHFVGRPMSTSSESSLIIKRLTLKLMQHSWSVSALTLDPAKLLEEFPRWLEKLSARHQGSIIIVIDSIDQVQQVEKHMKWLIDPLPVNVRVIVSVNVETCPPAWRLWPTLHLDPLSPKDAKSIIIAECHSVDIKLSKEQEKKLERHCRSATTCNALYVTLFGKMIARAGRAGNLDKILHQCFQCQDTLSLYRLVLHSIRESMANDVDKELMKQILCLVNVSHNGVSESELMELYPEMSWTFLTSLIHSLYKMCLLTYGCGLLRFQHLQAWETVRLEYLEGPTVTSSYRQKLINYFTLQLSQDRVTWRSADELPWLFQQQGSKQKLHDCLLNLFVSQNLYKRGHFAELLSYWQFVGKDKSAMATEYFDSLKQYEKNCEGEDNMSCLADLYETLGRFLKDLGLLSQAIVPLQRSLEIRETALDPDHPRVAQSLHQLASVYVQWKKFGNAEQLYKQALEISENAYGADHPYTARELEALATLYQKQNKYEQAEHFRKKSFKIHQKAIKKKGNLYGFALLRRRALQLEELTLGKDTPDNARTLNELGVLYYLQNNLETADQFLKRSLEMRERVLGPDHPDCAQSLNNLAALCNEKKQYDKAEELYERALDIRRRALAPDHPSLAYTVKHLAILYKKMGKLDKAVPLYELAVEIRQKSFGPKHPSVATALVNLAVLYSQMKKHVEALPLYERALKIYEDSLGRMHPRVGETLKNLAVLSYEGGDFEKAAELYKRAMEIKEAETSLLGGKAPSRHSSSGDTFSLKTAHSPNVFLQQGQR
Overview of Protein Modification Sites with Functional and Structural Information
Experimental Post-Translational Modification Sites

* ASA = Accessible Surface Area

Locations Modification Substrate Peptides
&
Secondary Structure		 ASA (%) Reference Orthologous
Protein Cluster
2Myristoylation------MGTASSLVS
------CCCHHHCCC		41.0922085962
2N-myristoyl glycine------MGTASSLVS
------CCCHHHCCC		41.09-
44PhosphorylationKARLLRNSFRRGAGA
CHHHHHHHHHCCCCC		17.4423403867
47MethylationLLRNSFRRGAGAAAG
HHHHHHHCCCCCCCC		36.9880701733
59PhosphorylationAAGAGPGSLPRGVGA
CCCCCCCCCCCCCCC		38.0324173317
73PhosphorylationAGGLLGASFKSTGSS
CCCCCCCCCCCCCCC		31.2628857561
121PhosphorylationRREAKLDTENKRLRA
CCCHHCCHHHHHHHH		52.47-
152PhosphorylationESALEAKYQAMERAA
HHHHHHHHHHHHHHH		14.4623403867
179UbiquitinationFKIFRETKENEIQDL
HHHHHHHCHHHHHHH		54.5129967540
441PhosphorylationAEGVYKTYICVEKII
CCCCHHHEEHHHHHH		6.5422210691
566PhosphorylationHFVGRPMSTSSESSL
EECCCCCCCCCCHHH		28.0223090842
567PhosphorylationFVGRPMSTSSESSLI
ECCCCCCCCCCHHHH		29.9823090842
568PhosphorylationVGRPMSTSSESSLII
CCCCCCCCCCHHHHH		25.4623090842
569PhosphorylationGRPMSTSSESSLIIK
CCCCCCCCCHHHHHH		41.4023090842
571PhosphorylationPMSTSSESSLIIKRL
CCCCCCCHHHHHHHH		32.1823090842
572PhosphorylationMSTSSESSLIIKRLT
CCCCCCHHHHHHHHH		21.3823090842
758PhosphorylationVLHSIRESMANDVDK
HHHHHHHHHCCHHCH		17.22-
916PhosphorylationQFVGKDKSAMATEYF
HHHCCCCHHHHHHHH		33.1528258704
920PhosphorylationKDKSAMATEYFDSLK
CCCHHHHHHHHHHHH		20.3828258704
925PhosphorylationMATEYFDSLKQYEKN
HHHHHHHHHHHHHHH		27.8028258704
969PhosphorylationAIVPLQRSLEIRETA
HHHHHHHHHHHHHHH		19.8624719451
987PhosphorylationDHPRVAQSLHQLASV
CCHHHHHHHHHHHHH		20.34-
1008PhosphorylationFGNAEQLYKQALEIS
HCCHHHHHHHHHHHH		10.76-
1039UbiquitinationALATLYQKQNKYEQA
HHHHHHHHCCHHHHH		42.1329967540
1095PhosphorylationDTPDNARTLNELGVL
CCCCCHHHHHHHHHH		31.37-
1158PhosphorylationYDKAEELYERALDIR
HHHHHHHHHHHHHHH		13.4729083192
1175PhosphorylationALAPDHPSLAYTVKH
HHCCCCHHHHHHHHH		23.3927174698
1178PhosphorylationPDHPSLAYTVKHLAI
CCCHHHHHHHHHHHH		19.8427174698
1179PhosphorylationDHPSLAYTVKHLAIL
CCHHHHHHHHHHHHH		19.9227174698
1187PhosphorylationVKHLAILYKKMGKLD
HHHHHHHHHHCCCCC		11.4918083107
1217PhosphorylationSFGPKHPSVATALVN
HCCCCCHHHHHHHHH		24.8922210691
1220PhosphorylationPKHPSVATALVNLAV
CCCHHHHHHHHHHHH		20.5422210691
1229PhosphorylationLVNLAVLYSQMKKHV
HHHHHHHHHHHHHHH		7.1122210691
1271PhosphorylationLKNLAVLSYEGGDFE
HHHHEEEEECCCCHH		17.9719690332
1272PhosphorylationKNLAVLSYEGGDFEK
HHHEEEEECCCCHHH		17.9019690332
1295PhosphorylationMEIKEAETSLLGGKA
HHHHHHHHHHCCCCC		31.5523532336
1307PhosphorylationGKAPSRHSSSGDTFS
CCCCCCCCCCCCCEE		25.7025850435
1308PhosphorylationKAPSRHSSSGDTFSL
CCCCCCCCCCCCEEE		32.0125850435
1309PhosphorylationAPSRHSSSGDTFSLK
CCCCCCCCCCCEEEE		43.6426699800
1317PhosphorylationGDTFSLKTAHSPNVF
CCCEEEEECCCCCCC		34.5923312004
1320PhosphorylationFSLKTAHSPNVFLQQ
EEEEECCCCCCCCCC		18.7325159151
Upstream regulatory proteins (kinases for phosphorylation sites, E3 ubiquitin ligases of ubiquitination sites, ...)
Modified Location Modified Residue Modification Type of Upstream Proteins Gene Name of Upstream Proteins UniProt AC of Upstream Proteins Sources

Oops, there are no upstream regulatory protein records of NPHP3_HUMAN !!
Functions of PTM Sites
Modified Location Modified Residue Modification Function Reference

Oops, there are no descriptions of PTM sites of NPHP3_HUMAN !!
Disease-associated PTM Sites based on SAP

* Distance = the distance between SAP position and PTM sites.

Modified Location Modification Variant Position
(Distance <= 10) 		Residue Change SAP Related Disease Reference

Oops, there are no SNP-PTM records of NPHP3_HUMAN !!
Protein-Protein Interaction
Interacting Protein Gene Name Interaction Type PPI Reference Domain-Domain Interactions
NPHP1_HUMANNPHP1physical
12872122
CE164_HUMANCEP164physical
22863007
ARG35_HUMANARHGEF35physical
28514442
U119B_HUMANUNC119Bphysical
28514442
GMDS_HUMANGMDSphysical
28514442
U119A_HUMANUNC119physical
27173435
OGT1_HUMANOGTphysical
27173435
PDE9A_HUMANPDE9Aphysical
27173435
OCC1_HUMANC12orf75physical
27173435
RSPRY_HUMANRSPRY1physical
27173435
FMNL2_HUMANFMNL2physical
27173435
PDE8A_HUMANPDE8Aphysical
27173435
KGP2_HUMANPRKG2physical
27173435
PALD_HUMANPALD1physical
27173435
ARL3_HUMANARL3physical
27173435
Drug and Disease Associations
Kegg Disease
There are no disease associations of PTM sites.
OMIM Disease
604387Nephronophthisis 3 (NPHP3)
208540Renal-hepatic-pancreatic dysplasia 1 (RHPD1)
267010Meckel syndrome 7 (MKS7)
Kegg Drug
There are no disease associations of PTM sites.
DrugBank
There are no disease associations of PTM sites.
Regulatory Network of NPHP3_HUMAN

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Related Literatures of Post-Translational Modification
Myristoylation
ReferencePubMed
"An ARL3-UNC119-RP2 GTPase cycle targets myristoylated NPHP3 to theprimary cilium.";
Wright K.J., Baye L.M., Olivier-Mason A., Mukhopadhyay S., Sang L.,Kwong M., Wang W., Pretorius P.R., Sheffield V.C., Sengupta P.,Slusarski D.C., Jackson P.K.;
Genes Dev. 25:2347-2360(2011).
Cited for: MYRISTOYLATION AT GLY-2, INTERACTION WITH UNC119 AND UNC119B, ANDSUBCELLULAR LOCATION.

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