Gene Symbol: nphp-1
Description: Nephrocystin-1-like protein
Alias: Nephrocystin-1-like protein
Species: Caenorhabditis elegans

Top Publications

  1. Wolf M, Lee J, Panther F, Otto E, Guan K, Hildebrandt F. Expression and phenotype analysis of the nephrocystin-1 and nephrocystin-4 homologs in Caenorhabditis elegans. J Am Soc Nephrol. 2005;16:676-87 pubmed
    ..The NPHP homologs may be necessary for initial assembly of the cilium, whereas the polycystic kidney disease homologs may function as sensory transducers. ..
  2. Jauregui A, Barr M. Functional characterization of the C. elegans nephrocystins NPHP-1 and NPHP-4 and their role in cilia and male sensory behaviors. Exp Cell Res. 2005;305:333-42 pubmed
    ..nphp-1; nphp-4 double, but not single, mutant males are response defective. We propose that NPHP-1 and NPHP-4 proteins play important and redundant roles in facilitating ciliary sensory signal transduction. ..
  3. Jauregui A, Nguyen K, Hall D, Barr M. The Caenorhabditis elegans nephrocystins act as global modifiers of cilium structure. J Cell Biol. 2008;180:973-88 pubmed publisher
  4. Williams C, Winkelbauer M, Schafer J, Michaud E, Yoder B. Functional redundancy of the B9 proteins and nephrocystins in Caenorhabditis elegans ciliogenesis. Mol Biol Cell. 2008;19:2154-68 pubmed publisher
    ..Together, these data suggest that the human homologues of the novel B9 genes B9D2 and B9D1 will be strong candidate loci for pathologies in human MKS, NPHP, and JBTS. ..
  5. Bialas N, Inglis P, Li C, Robinson J, Parker J, Healey M, et al. Functional interactions between the ciliopathy-associated Meckel syndrome 1 (MKS1) protein and two novel MKS1-related (MKSR) proteins. J Cell Sci. 2009;122:611-24 pubmed publisher
    ..Our findings therefore demonstrate functional interactions between a novel family of proteins associated with basal bodies or cilia, providing new insights into the molecular etiology of a pleiotropic human disorder. ..
  6. Williams C, Li C, Kida K, Inglis P, Mohan S, Semenec L, et al. MKS and NPHP modules cooperate to establish basal body/transition zone membrane associations and ciliary gate function during ciliogenesis. J Cell Biol. 2011;192:1023-41 pubmed publisher
  7. Nguyen P, Liou W, Hall D, Leroux M. Ciliopathy proteins establish a bipartite signaling compartment in a C. elegans thermosensory neuron. J Cell Sci. 2014;127:5317-30 pubmed publisher
    ..We propose that differential compartmentalization of signal transduction components by ciliary proteins is important for the functions of ciliated sensory neurons. ..
  8. Lambacher N, Bruel A, van Dam T, Szymańska K, Slaats G, Kuhns S, et al. TMEM107 recruits ciliopathy proteins to subdomains of the ciliary transition zone and causes Joubert syndrome. Nat Cell Biol. 2016;18:122-31 pubmed publisher
    ..This work expands the MKS module of ciliopathy-causing TZ proteins associated with diffusion barrier formation and provides insight into TZ subdomain architecture. ..