نتایج جستجو برای: pkd2

تعداد نتایج: 596  

2015
Qingwen Xu Yuxia Zhang Qing Wei Yan Huang Yan Li Kun Ling Jinghua Hu

Cilia harbor sensory receptors for various signaling cascades critical for vertebrate development. However, the mechanisms underlying the ciliary homeostasis of sensory receptors remain elusive. Here, we demonstrate that BBS-4 and BBS-5, two distinct BBSome components, show unexpected functional redundancy in the context of cilia in C. elegans. BBS-4 directly interacts with BBS-5 and the intera...

2015
Elle C. Roberson William E. Dowdle Aysegul Ozanturk Francesc R. Garcia-Gonzalo Chunmei Li Jan Halbritter Nadia Elkhartoufi Jonathan D. Porath Heidi Cope Allison Ashley-Koch Simon Gregory Sophie Thomas John A. Sayer Sophie Saunier Edgar A. Otto Nicholas Katsanis Erica E. Davis Tania Attié-Bitach Friedhelm Hildebrandt Michel R. Leroux Jeremy F. Reiter

The Rockefeller University Press $30.00 J. Cell Biol. Vol. 209 No. 1 129–142 www.jcb.org/cgi/doi/10.1083/jcb.201411087 JCB 129 *E.C. Roberson and W.E. Dowdle contributed equally to this paper. Correspondence to Jeremy F. Reiter: [email protected] Abbreviations used in this paper: Adcy3, Adenylyl cyclase 3; E, embryonic day; LAP, localization and affinity purification; MEF, mouse embryonic ...

2014

Grants to the second and third authors (AV and JP) are incorrectly omitted from the Funding statement. The Funding statement should read: ‘‘The PC lab was supported by the Swiss National Foundation for Scientific Research (grant 31003A135789), the Doerenkamp-Zbinden Foundation and the Fondation Egon Naef pour la Recherche in Vitro. The JP lab was supported by grants from the Swiss National Scie...

Journal: :PloS one 2015
Lisa Te Molder Arnoud Sonnenberg

The integrin α6β4, a major component of hemidesmosomes (HDs), stabilizes keratinocyte cell adhesion to the epidermal basement membrane through binding to the cytoskeletal linker protein plectin and association with keratin filaments. Disruption of the α6β4-plectin interaction through phosphorylation of the β4 subunit results in a reduction in adhesive strength of keratinocytes to laminin-332 an...

Journal: :Experimental cell research 2005
Andrew R Jauregui Maureen M Barr

Autosomal dominant polycystic kidney disease (ADPKD) and nephronophthisis (NPH) share two common features: cystic kidneys and ciliary localized gene products. Mutation in either the PKD1 or PKD2 gene accounts for 95% of all ADPKD cases. Mutation in one of four genes (NPHP1-4) results in nephronophthisis. The NPHP1, NPHP2, PKD1, and PKD2 protein products (nephrocystin-1, nephrocystin-2 or invers...

Journal: :The Journal of clinical investigation 2002
Christian Nickel Thomas Benzing Lorenz Sellin Peter Gerke Anil Karihaloo Zhen-Xiang Liu Lloyd G Cantley Gerd Walz

Mutations of either PKD1 or PKD2 cause autosomal dominant polycystic kidney disease, a syndrome characterized by extensive formation of renal cysts and progressive renal failure. Homozygous deletion of Pkd1 or Pkd2, the genes encoding polycystin-1 and polycystin-2, disrupt normal renal tubular differentiation in mice but do not affect the early steps of renal development. Here, we show that exp...

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