CPLM 1.0 - Compendium of Protein Lysine Modification
TagContent
CPLM ID CPLM-023619
UniProt Accession
Genbank Protein ID
Genbank Nucleotide ID
Protein Name
 Inversin 
Protein Synonyms/Alias
 Inversion of embryo turning homolog; Nephrocystin-2 
Gene Name
 INVS 
Gene Synonyms/Alias
 INV; NPHP2 
Created Date
 July 27, 2013 
Organism
 Homo sapiens (Human) 
NCBI Taxa ID
 9606 
Lysine Modification
Position
Peptide
Type
References
110RRANWMQKDLEEMTPubiquitination[1]
170EHVKLLIKHDSNIGIubiquitination[1]
310ETVKVFLKHPSVKDDubiquitination[1]
332SFMWAAGKGSDDVLRubiquitination[1]
390VDATDVMKHTPLFRAubiquitination[1, 2, 3]
403RACEMGHKDVIQTLIubiquitination[1]
Reference
 [1] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961]
 [2] A proteome-wide, quantitative survey of in vivo ubiquitylation sites reveals widespread regulatory roles.
 Wagner SA, Beli P, Weinert BT, Nielsen ML, Cox J, Mann M, Choudhary C.
 Mol Cell Proteomics. 2011 Oct;10(10):M111.013284. [PMID: 21890473]
 [3] hCKSAAP_UbSite: improved prediction of human ubiquitination sites by exploiting amino acid pattern and properties.
 Chen Z, Zhou Y, Song J, Zhang Z.
 Biochim Biophys Acta. 2013 Aug;1834(8):1461-7. [PMID: 23603789] 
Functional Description
 Required for normal renal development and establishment of left-right axis. Probably acts as a molecular switch between different Wnt signaling pathways. Inhibits the canonical Wnt pathway by targeting cytoplasmic disheveled (DVL1) for degradation by the ubiquitin-proteasome. This suggests that it is required in renal development to oppose the repression of terminal differentiation of tubular epithelial cells by Wnt signaling. Involved in the organization of apical junctions in kidney cells together with NPHP1, NPHP4 and RPGRIP1L/NPHP8 (By similarity). Does not seem to be strictly required for ciliogenesis (By similarity). 
Sequence Annotation
 REPEAT 13 42 ANK 1.
 REPEAT 47 76 ANK 2.
 REPEAT 80 110 ANK 3.
 REPEAT 113 144 ANK 4.
 REPEAT 148 177 ANK 5.
 REPEAT 181 213 ANK 6.
 REPEAT 220 250 ANK 7.
 REPEAT 254 283 ANK 8.
 REPEAT 288 317 ANK 9.
 REPEAT 321 350 ANK 10.
 REPEAT 356 385 ANK 11.
 REPEAT 389 418 ANK 12.
 REPEAT 422 451 ANK 13.
 REPEAT 455 484 ANK 14.
 REPEAT 488 517 ANK 15.
 REPEAT 523 553 ANK 16.
 DOMAIN 555 584 IQ 1.
 DOMAIN 916 945 IQ 2.
 MOTIF 490 498 D-box 1.
 MOTIF 909 917 D-box 2.  
Keyword
 Alternative splicing; ANK repeat; Calmodulin-binding; Cell projection; Ciliopathy; Cilium; Complete proteome; Cytoplasm; Cytoskeleton; Developmental protein; Disease mutation; Membrane; Microtubule; Nephronophthisis; Nucleus; Reference proteome; Repeat; Ubl conjugation; Wnt signaling pathway. 
Sequence Source
 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 
Protein Length
 1065 AA 
Protein Sequence
MNKSENLLFA GSSLASQVHA AAVNGDKGAL QRLIVGNSAL KDKEDQFGRT PLMYCVLADR 60
LDCADALLKA GADVNKTDHS QRTALHLAAQ KGNYRFMKLL LTRRANWMQK DLEEMTPLHL 120
TTRHRSPKCL ALLLKFMAPG EVDTQDKNKQ TALHWSAYYN NPEHVKLLIK HDSNIGIPDV 180
EGKIPLHWAA NHKDPSAVHT VRCILDAAPT ESLLNWQDYE GRTPLHFAVA DGNVTVVDVL 240
TSYESCNITS YDNLFRTPLH WAALLGHAQI VHLLLERNKS GTIPSDSQGA TPLHYAAQSN 300
FAETVKVFLK HPSVKDDSDL EGRTSFMWAA GKGSDDVLRT MLSLKSDIDI NMADKYGGTA 360
LHAAALSGHV STVKLLLENN AQVDATDVMK HTPLFRACEM GHKDVIQTLI KGGARVDLVD 420
QDGHSLLHWA ALGGNADVCQ ILIENKINPN VQDYAGRTPL QCAAYGGYIN CMAVLMENNA 480
DPNIQDKEGR TALHWSCNNG YLDAIKLLLD FAAFPNQMEN NEERYTPLDY ALLGERHEVI 540
QFMLEHGALS IAAIQDIAAF KIQAVYKGYK VRKAFRDRKN LLMKHEQLRK DAAAKKREEE 600
NKRKEAEQQK GRRSPDSCRP QALPCLPSTQ DVPSRQSRAP SKQPPAGNVA QGPEPRDSRG 660
SPGGSLGGAL QKEQHVSSDL QGTNSRRPNE TAREHSKGQS ACVHFRPNEG SDGSRHPGVP 720
SVEKSRGETA GDERCAKGKG FVKQPSCIRV AGPDEKGEDS RRAAASLPPH DSHWKPSRRH 780
DTEPKAKCAP QKRRTQELRG GRCSPAGSSR PGSARGEAVH AGQNPPHHRT PRNKVTQAKL 840
TGGLYSHLPQ STEELRSGAR RLETSTLSED FQVSKETDPA PGPLSGQSVN IDLLPVELRL 900
QIIQRERRRK ELFRKKNKAA AVIQRAWRSY QLRKHLSHLR HMKQLGAGDV DRWRQESTAL 960
LLQVWRKELE LKFPQTTAVS KAPKSPSKGT SGTKSTKHSV LKQIYGCSHE GKIHHPTRSV 1020
KASSVLRLNS VSNLQCIHLL ENSGRSKNFS YNLQSATQPK NKTKP 1065 
Gene Ontology
 GO:0005929; C:cilium; IEA:UniProtKB-SubCell.
 GO:0005737; C:cytoplasm; IEA:UniProtKB-SubCell.
 GO:0016020; C:membrane; IEA:UniProtKB-SubCell.
 GO:0005874; C:microtubule; IEA:UniProtKB-KW.
 GO:0005634; C:nucleus; IEA:UniProtKB-SubCell.
 GO:0005819; C:spindle; IEA:UniProtKB-SubCell.
 GO:0060971; P:embryonic heart tube left/right pattern formation; IEA:Compara.
 GO:0001822; P:kidney development; IEA:Compara.
 GO:0090090; P:negative regulation of canonical Wnt receptor signaling pathway; IDA:UniProtKB.
 GO:0031016; P:pancreas development; IEA:Compara.
 GO:0009791; P:post-embryonic development; IEA:Compara.
 GO:0016055; P:Wnt receptor signaling pathway; IEA:UniProtKB-KW. 
Interpro
 IPR002110; Ankyrin_rpt.
 IPR020683; Ankyrin_rpt-contain_dom.
 IPR000048; IQ_motif_EF-hand-BS. 
Pfam
 PF12796; Ank_2
 PF00612; IQ 
SMART
 SM00248; ANK
 SM00015; IQ 
PROSITE
 PS50297; ANK_REP_REGION
 PS50088; ANK_REPEAT
 PS50096; IQ 
PRINTS
 PR01415; ANKYRIN.