Nephronophthisis and Wnt Signaling (Mus musculus)

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Canonical Cytoskeleton/planar cell Polarity Non-Canonical Transcription Calcium Rhoa beta-catenin Nphp3 Nphp2 (Invs) Lrp5 Lrp6 Fzd1 Fzd8 Fzd4 Fzd2 Fzd7 Fzd9 Fzd5 Fzd6 Fzd3 Wnt1 Wnt10a Wnt10b Wnt7a Wnt16 Wnt6 Wnt5a Wnt2b Wnt11 Wnt4 Wnt3a Wnt5b Wnt9b Wnt2 Wnt3 Wnt7b Fzd1 Fzd8 Fzd4 Fzd2 Fzd7 Fzd9 Fzd5 Fzd6 Fzd3 Dvl2 Dvl3 Dvl1 Mapk9 Mapk10 Mapk8 Dvl2 Dvl3 Dvl1 cytoplasmic disheveled membrane-bound disheveled Name: Wnt Signaling in Kidney Disease Organism: Mus musculus


This pathway is modeled after Figure 5 of the article "Glomerulocystic kidney disease" (Bissler, et al. 2010). The gene product Wnt signals two different paths titled canonical and non-canonical. The non-canonical path is mediated by the membrane bound protein Disheveled (Dvl) which then controls the planar cell Polarity. The canonical path on the other hand is mediated by the cytoplasmic protein Disheveled (Dvl) and then is effected through b-catenin transcription. The complex of Nphp2 and Nphp3 seem to help facilitate the process by controlling the Wnt signaling. Bergmann, et al. (2008) showed that Nphp3 deficiency in Xenopus resulted to planar cell polarity defects.

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Ontology Terms



  1. Bergmann C, Fliegauf M, Brüchle NO, Frank V, Olbrich H, Kirschner J, Schermer B, Schmedding I, Kispert A, Kränzlin B, Nürnberg G, Becker C, Grimm T, Girschick G, Lynch SA, Kelehan P, Senderek J, Neuhaus TJ, Stallmach T, Zentgraf H, Nürnberg P, Gretz N, Lo C, Lienkamp S, Schäfer T, Walz G, Benzing T, Zerres K, Omran H; ''Loss of nephrocystin-3 function can cause embryonic lethality, Meckel-Gruber-like syndrome, situs inversus, and renal-hepatic-pancreatic dysplasia.''; Am J Hum Genet, 2008 PubMed
  2. Bissler JJ, Siroky BJ, Yin H; ''Glomerulocystic kidney disease.''; Pediatr Nephrol, 2010 PubMed


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90797view 10:25, 14 December 2016KhanspersModified title
90183view 11:28, 21 October 2016Khanspersupdated lit ref
88437view 14:20, 6 August 2016AlexanderPicoUpdated references
88436view 14:19, 6 August 2016AlexanderPicoModified description
88435view 14:11, 6 August 2016AlexanderPicoUpdated title, lit ref and label
88434view 14:02, 6 August 2016AlexanderPicoOntology Term : 'signaling pathway' added !
88433view 14:00, 6 August 2016AlexanderPicoAdded paralogs, Lrp5/6, cell compartments and Dvl labels
88380view 18:21, 1 August 2016AAR&CoOntology Term : 'Wnt signaling, the planar cell polarity pathway' added !
88379view 18:20, 1 August 2016AAR&CoOntology Term : 'kidney disease' added !
88217view 17:37, 31 July 2016AAR&CoAdded Identifier
88216view 17:35, 31 July 2016AAR&CoAdded Identifiers
87416view 04:31, 22 July 2016MkutmonOntology Term : 'Wnt signaling pathway' added !
87279view 09:47, 20 July 2016AAR&CoIdentifiers Added
87277view 09:43, 20 July 2016AAR&CoModified description
87268view 09:08, 20 July 2016AAR&CoNew pathway

External references


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NameTypeDatabase referenceComment
CalciumMetaboliteHMDB00464 (HMDB)
Dvl1GeneProduct13542 (Entrez Gene)
Dvl2GeneProduct13543 (Entrez Gene)
Dvl3GeneProduct13544 (Entrez Gene)
Fzd1GeneProduct14362 (Entrez Gene)
Fzd2GeneProduct57265 (Entrez Gene)
Fzd3GeneProduct14365 (Entrez Gene)
Fzd4GeneProduct14366 (Entrez Gene)
Fzd5GeneProduct14367 (Entrez Gene)
Fzd6GeneProduct14368 (Entrez Gene)
Fzd7GeneProduct14369 (Entrez Gene)
Fzd8GeneProduct14370 (Entrez Gene)
Fzd9GeneProduct14371 (Entrez Gene)
Lrp5GeneProductENSMUSG00000024913 (Ensembl)
Lrp6GeneProductENSMUSG00000030201 (Ensembl)
Mapk10GeneProduct26414 (Entrez Gene)
Mapk8GeneProduct26419 (Entrez Gene)
Mapk9GeneProduct26420 (Entrez Gene)
Nphp2 (Invs)GeneProductENSMUSG00000028344 (Ensembl)
Nphp3GeneProductENSMUSG00000032558 (Ensembl)
RhoaGeneProductENSMUSG00000007815 (Ensembl)
Wnt10aGeneProduct22409 (Entrez Gene)
Wnt10bGeneProduct22410 (Entrez Gene)
Wnt11GeneProduct22411 (Entrez Gene)
Wnt1GeneProduct22408 (Entrez Gene)
Wnt16GeneProduct93735 (Entrez Gene)
Wnt2GeneProduct22413 (Entrez Gene)
Wnt2bGeneProduct22414 (Entrez Gene)
Wnt3GeneProduct22415 (Entrez Gene)
Wnt3aGeneProduct22416 (Entrez Gene)
Wnt4GeneProduct22417 (Entrez Gene)
Wnt5aGeneProduct22418 (Entrez Gene)
Wnt5bGeneProduct22419 (Entrez Gene)
Wnt6GeneProduct22420 (Entrez Gene)
Wnt7aGeneProduct22421 (Entrez Gene)
Wnt7bGeneProduct22422 (Entrez Gene)
Wnt9bGeneProduct22412 (Entrez Gene)
beta-cateninGeneProductENSMUSG00000006932 (Ensembl)

Annotated Interactions

<cite>No annotated interactions</cite>