JCB logo
Accuri Cytometers
  Home | Help | Feedback | Subscriptions | Archive | Search | Table of Contents
Published 23 July 2001. doi:10.1083/jcb.200104083
This Article
Right arrow Full Text
Right arrow Full Text (PDF, 727K)
Right arrow PPT slides of all figures
Right arrow Alert me when this article is cited
Right arrow Citation Map
Services
Right arrow Email this article
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new content in the JCB
Right arrow Download to citation manager
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Tucker, K. E.
Right arrow Articles by Matera, A. G.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Tucker, K. E.
Right arrow Articles by Matera, A. G.
Social Bookmarking
Add to CiteULike   Add to Complore   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Facebook   Add to Reddit   Add to Technorati   Add to Twitter  
What's this?

© The Rockefeller University Press, 0021-9525/2001/7/293 $5.00
The Journal of Cell Biology, Volume 154, Number 2, July 23, 2001 293-308

Article

Residual Cajal bodies in coilin knockout mice fail to recruit Sm snRNPs and SMN, the spinal muscular atrophy gene product



Karen E. Tucker1,2, Maria Teresa Berciano3, Erica Y. Jacobs1,2, David F. LePage1, Karl B. Shpargel1,2, Jennifer J. Rossire1,2, Edward K.L. Chan4, Miguel Lafarga3, Ronald A. Conlon1 and A. Gregory Matera1,2

1 Department of Genetics, Case Western Reserve University and University Hospitals of Cleveland, Cleveland, OH 44106
2 Program in Cell Biology, Case Western Reserve University and University Hospitals of Cleveland, Cleveland, OH 44106
3 Department of Anatomy and Cell Biology, Faculty of Medicine, University of Cantabria, 39011 Santander, Spain
4 W.M. Keck Autoimmune Disease Center, The Scripps Research Institute, La Jolla, CA 92037

Address correspondence to Gregory Matera, Dept. of Genetics, Case Western Reserve University, 10900 Euclid Ave., Cleveland, OH 44106-4955. Tel.: (216) 368-4922. Fax: (216) 368-3432. E-mail: gxm26{at}po.cwru.edu

Cajal bodies (CBs) are nuclear suborganelles involved in the biogenesis of small nuclear ribonucleoproteins (snRNPs). In addition to snRNPs, they are highly enriched in basal transcription and cell cycle factors, the nucleolar proteins fibrillarin (Fb) and Nopp140 (Nopp), the survival motor neuron (SMN) protein complex, and the CB marker protein, p80 coilin. We report the generation of knockout mice lacking the COOH-terminal 487 amino acids of coilin. Northern and Western blot analyses demonstrate that we have successfully removed the full-length coilin protein from the knockout animals. Some homozygous mutant animals are viable, but their numbers are reduced significantly when crossed to inbred backgrounds. Analysis of tissues and cell lines from mutant animals reveals the presence of extranucleolar foci that contain Fb and Nopp but not other typical nucleolar markers. These so-called "residual" CBs neither condense Sm proteins nor recruit members of the SMN protein complex. Transient expression of wild-type mouse coilin in knockout cells results in formation of CBs and restores these missing epitopes. Our data demonstrate that full-length coilin is essential for proper formation and/or maintenance of CBs and that recruitment of snRNP and SMN complex proteins to these nuclear subdomains requires sequences within the coilin COOH terminus.

Key Words: coilin; SMN; SMA; snRNPs; nuclear organization


Add to CiteULike CiteULike   Add to Complore Complore   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Facebook Facebook   Add to Reddit Reddit   Add to Technorati Technorati   Add to Twitter Twitter    What's this?


This article has been cited by other articles:



  Home | Help | Feedback | Subscriptions | Archive | Search | Table of Contents