JCB logo
PeproTech: Cell Culture Supplements
  Home | Help | Feedback | Subscriptions | Archive | Search | Table of Contents
This Article
Right arrow Full Text
Right arrow Full Text (PDF, 1178K)
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 Costell, M.
Right arrow Articles by Fässler, R.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Costell, M.
Right arrow Articles by Fässler, R.
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, 0021-9525/1999//1109 $5.00
The Journal of Cell Biology, Volume 147, Number 5, , 1999 1109-1122

Original Article

Perlecan Maintains the Integrity of Cartilage and Some Basement Membranes



Mercedes Costella,b,c, Erika Gustafssona, Attila Aszódia, Matthias Mörgelina, Wilhelm Blochd, Ernst Hunzikere, Klaus Addicksd, Rupert Timplb, and Reinhard Fässlera

a Department of Experimental Pathology, Lund University, S-221 85 Lund, Sweden
b Max Planck Institute for Biochemistry, 82152 Martinsried, Germany
c Department of Biochemistry and Molecular Biology, University of Valencia, 46071 Valencia, Spain
d Institute for Anatomy, University of Cologne, 50931 Cologne, Germany
e M.E. Müller Institute for Biomechanics, University of Bern, 3010 Bern, Switzerland
Department of Experimental Pathology, Lund University, S-221 85 Lund, Sweden.46-46-15820246-46-173400

reinhard.fassler{at}pat.lu.se

Perlecan is a heparan sulfate proteoglycan that is expressed in all basement membranes (BMs), in cartilage, and several other mesenchymal tissues during development. Perlecan binds growth factors and interacts with various extracellular matrix proteins and cell adhesion molecules. Homozygous mice with a null mutation in the perlecan gene exhibit normal formation of BMs. However, BMs deteriorate in regions with increased mechanical stress such as the contracting myocardium and the expanding brain vesicles showing that perlecan is crucial for maintaining BM integrity. As a consequence, small clefts are formed in the cardiac muscle leading to blood leakage into the pericardial cavity and an arrest of heart function. The defects in the BM separating the brain from the adjacent mesenchyme caused invasion of brain tissue into the overlaying ectoderm leading to abnormal expansion of neuroepithelium, neuronal ectopias, and exencephaly. Finally, homozygotes developed a severe defect in cartilage, a tissue that lacks BMs. The chondrodysplasia is characterized by a reduction of the fibrillar collagen network, shortened collagen fibers, and elevated expression of cartilage extracellular matrix genes, suggesting that perlecan protects cartilage extracellular matrix from degradation.

Key Words: perlecan • basement membrane • cardiac muscle • exencephaly • chondrodysplasia

© 1999 The Rockefeller University Press

Abbreviations used in this paper: Aβ, β amyloid; AD, Alzheimer's disease; BMs, basement membranes; ECM, extracellular matrix; ES, embryonic stem; RT-PCR, reverse transcriptase–PCR.


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