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Perlecan maintains the integrity of cartilage and some basement membranes

Costell, Mercedes; Gustafsson, Erika LU ; Aszodi, Attila LU ; Mörgelin, Matthias LU ; Bloch, W; Hunziker, E; Addicks, K; Timpl, R and Fässler, Reinhard LU (1999) In Journal of Cell Biology 147(5). p.1109-1122
Abstract
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... (More)
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. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
chondrodysplasia, exencephaly, cardiac muscle, perlecan, basement membrane
in
Journal of Cell Biology
volume
147
issue
5
pages
1109 - 1122
publisher
Rockefeller University Press
external identifiers
  • pmid:10579729
  • scopus:0033615959
ISSN
0021-9525
language
English
LU publication?
yes
id
e92e1fe8-e581-4863-945a-054b16390afc (old id 1115452)
alternative location
http://www.jcb.org/cgi/content/full/147/5/1109
date added to LUP
2008-07-08 09:44:47
date last changed
2017-07-30 03:38:29
@article{e92e1fe8-e581-4863-945a-054b16390afc,
  abstract     = {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.},
  author       = {Costell, Mercedes and Gustafsson, Erika and Aszodi, Attila and Mörgelin, Matthias and Bloch, W and Hunziker, E and Addicks, K and Timpl, R and Fässler, Reinhard},
  issn         = {0021-9525},
  keyword      = {chondrodysplasia,exencephaly,cardiac muscle,perlecan,basement membrane},
  language     = {eng},
  number       = {5},
  pages        = {1109--1122},
  publisher    = {Rockefeller University Press},
  series       = {Journal of Cell Biology},
  title        = {Perlecan maintains the integrity of cartilage and some basement membranes},
  volume       = {147},
  year         = {1999},
}