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Gene Review

Pcdh1  -  protocadherin 1

Mus musculus

Synonyms: 2010005A06Rik, AI585920
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Disease relevance of Pcdh1


High impact information on Pcdh1

  • Here we report that the gene that harbours the av mutation encodes a novel protocadherin [1].
  • This is the first evidence for the requirement of a protocadherin for normal function of the mammalian inner ear [1].
  • Recent progress in genome sequencing has enabled a refined phylogenetic analysis of protocadherins and led to the discovery of three large protocadherin clusters on human chromosome 5/mouse chromosome 18 [5].
  • Despite the extensive analyses of the genomic structure of both human and mouse Pcdh gene clusters, the definitive molecular mechanisms that control Pcdh gene expression are still unknown [6].
  • Molecular mechanisms governing Pcdh-gamma gene expression: evidence for a multiple promoter and cis-alternative splicing model [6].

Chemical compound and disease context of Pcdh1


Biological context of Pcdh1

  • Mouse Pcdh1 and Pcdh2 were mapped to a small segment of chromosome 18, suggesting that various protocadherins form a gene cluster at this region [8].
  • To study the diversity of the protocadherin family, the cDNA clones for a novel protocadherin were isolated by screening rat brain cDNA libraries with a cDNA fragment obtained by PCR, and some of the properties were then characterized [9].
  • Multiple alpha and gamma Pcdh mRNAs are expressed in distinct patterns in the nervous system and are generated by alternative pre-mRNA splicing between different "variable" exons and three "constant" exons within each cluster [10].
  • The murine genome contains approximately 70 protocadherin (Pcdh) genes [11].
  • Previous work using tools of Drosophila genetics suggested that protocadherin fat serves in a pathway necessary for determining cell polarity in the plane of a tissue [12].

Anatomical context of Pcdh1


Associations of Pcdh1 with chemical compounds


Regulatory relationships of Pcdh1


Other interactions of Pcdh1

  • We named this protocad herin-3 (Pcdh3) since this is the third protocadherin of which the entire coding sequence has been determined [9].
  • This subfamily can be further subdivided into two subgroups, named delta1-protocadherins (comprising protocadherin-1, -7, -9, and -11 or X/Y) and delta2-protocadherins (comprising protocadherin-8, -10, -17, -18, and -19) [17].
  • In contrast, the expression of EphA4 (Epha4), lunatic fringe or protocadherin, thought to be involved in segment border formation, was fairly normal in hypomorphic mutant embryos [18].
  • We show that these mutants, spin cycle and crash, carry independent missense mutations within the coding region of Celsr1, encoding a large protocadherin molecule [1] [19].
  • By linkage analyses, the mutant phenotype was mapped near marker D10Mit186 and the protocadherin gene Pcdh15 [20].

Analytical, diagnostic and therapeutic context of Pcdh1


  1. The mouse Ames waltzer hearing-loss mutant is caused by mutation of Pcdh15, a novel protocadherin gene. Alagramam, K.N., Murcia, C.L., Kwon, H.Y., Pawlowski, K.S., Wright, C.G., Woychik, R.P. Nat. Genet. (2001) [Pubmed]
  2. Mutations of the protocadherin gene PCDH15 cause Usher syndrome type 1F. Ahmed, Z.M., Riazuddin, S., Bernstein, S.L., Ahmed, Z., Khan, S., Griffith, A.J., Morell, R.J., Friedman, T.B., Riazuddin, S., Wilcox, E.R. Am. J. Hum. Genet. (2001) [Pubmed]
  3. Mice lacking the giant protocadherin mFAT1 exhibit renal slit junction abnormalities and a partially penetrant cyclopia and anophthalmia phenotype. Ciani, L., Patel, A., Allen, N.D., ffrench-Constant, C. Mol. Cell. Biol. (2003) [Pubmed]
  4. The emergence of protocadherin-PC expression during the acquisition of apoptosis-resistance by prostate cancer cells. Chen, M.W., Vacherot, F., De La Taille, A., Gil-Diez-De-Medina, S., Shen, R., Friedman, R.A., Burchardt, M., Chopin, D.K., Buttyan, R. Oncogene (2002) [Pubmed]
  5. Protocadherins. Frank, M., Kemler, R. Curr. Opin. Cell Biol. (2002) [Pubmed]
  6. Molecular mechanisms governing Pcdh-gamma gene expression: evidence for a multiple promoter and cis-alternative splicing model. Wang, X., Su, H., Bradley, A. Genes Dev. (2002) [Pubmed]
  7. Protocadherin-PC promotes androgen-independent prostate cancer cell growth. Terry, S., Queires, L., Gil-Diez-de-Medina, S., Chen, M.W., de la Taille, A., Allory, Y., Tran, P.L., Abbou, C.C., Buttyan, R., Vacherot, F. Prostate (2006) [Pubmed]
  8. Protocadherin Pcdh2 shows properties similar to, but distinct from, those of classical cadherins. Obata, S., Sago, H., Mori, N., Rochelle, J.M., Seldin, M.F., Davidson, M., St John, T., Taketani, S., Suzuki, S.T. J. Cell. Sci. (1995) [Pubmed]
  9. Cloning, expression, and chromosomal localization of a novel cadherin-related protein, protocadherin-3. Sago, H., Kitagawa, M., Obata, S., Mori, N., Taketani, S., Rochelle, J.M., Seldin, M.F., Davidson, M., St John, T., Suzuki, S.T. Genomics (1995) [Pubmed]
  10. Promoter choice determines splice site selection in protocadherin alpha and gamma pre-mRNA splicing. Tasic, B., Nabholz, C.E., Baldwin, K.K., Kim, Y., Rueckert, E.H., Ribich, S.A., Cramer, P., Wu, Q., Axel, R., Maniatis, T. Mol. Cell (2002) [Pubmed]
  11. Gamma protocadherins are required for survival of spinal interneurons. Wang, X., Weiner, J.A., Levi, S., Craig, A.M., Bradley, A., Sanes, J.R. Neuron (2002) [Pubmed]
  12. Protocadherin FAT1 binds Ena/VASP proteins and is necessary for actin dynamics and cell polarization. Moeller, M.J., Soofi, A., Braun, G.S., Li, X., Watzl, C., Kriz, W., Holzman, L.B. EMBO J. (2004) [Pubmed]
  13. Gamma protocadherins are required for synaptic development in the spinal cord. Weiner, J.A., Wang, X., Tapia, J.C., Sanes, J.R. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  14. PCNS: a novel protocadherin required for cranial neural crest migration and somite morphogenesis in Xenopus. Rangarajan, J., Luo, T., Sargent, T.D. Dev. Biol. (2006) [Pubmed]
  15. Binding of purified Reelin to ApoER2 and VLDLR mediates tyrosine phosphorylation of Disabled-1. Benhayon, D., Magdaleno, S., Curran, T. Brain Res. Mol. Brain Res. (2003) [Pubmed]
  16. Interaction with protocadherin-gamma regulates the cell surface expression of protocadherin-alpha. Murata, Y., Hamada, S., Morishita, H., Mutoh, T., Yagi, T. J. Biol. Chem. (2004) [Pubmed]
  17. delta-Protocadherins: a gene family expressed differentially in the mouse brain. Vanhalst, K., Kools, P., Staes, K., van Roy, F., Redies, C. Cell. Mol. Life Sci. (2005) [Pubmed]
  18. Hypomorphic Mesp allele distinguishes establishment of rostrocaudal polarity and segment border formation in somitogenesis. Nomura-Kitabayashi, A., Takahashi, Y., Kitajima, S., Inoue, T., Takeda, H., Saga, Y. Development (2002) [Pubmed]
  19. Mutation of Celsr1 disrupts planar polarity of inner ear hair cells and causes severe neural tube defects in the mouse. Curtin, J.A., Quint, E., Tsipouri, V., Arkell, R.M., Cattanach, B., Copp, A.J., Henderson, D.J., Spurr, N., Stanier, P., Fisher, E.M., Nolan, P.M., Steel, K.P., Brown, S.D., Gray, I.C., Murdoch, J.N. Curr. Biol. (2003) [Pubmed]
  20. A new spontaneous mutation in the mouse Ames waltzer gene, Pcdh15. Hampton, L.L., Wright, C.G., Alagramam, K.N., Battey, J.F., Noben-Trauth, K. Hear. Res. (2003) [Pubmed]
  21. Mutations in the novel protocadherin PCDH15 cause Usher syndrome type 1F. Alagramam, K.N., Yuan, H., Kuehn, M.H., Murcia, C.L., Wayne, S., Srisailpathy, C.R., Lowry, R.B., Knaus, R., Van Laer, L., Bernier, F.P., Schwartz, S., Lee, C., Morton, C.C., Mullins, R.F., Ramesh, A., Van Camp, G., Hageman, G.S., Woychik, R.P., Smith, R.J., Hagemen, G.S. Hum. Mol. Genet. (2001) [Pubmed]
  22. Sequence analysis and expression mapping of the rat clustered protocadherin gene repertoires. Zou, C., Huang, W., Ying, G., Wu, Q. Neuroscience (2007) [Pubmed]
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