Dec 1, 1999

Perlecan maintains the integrity of cartilage and some basement membranes

The Journal of Cell Biology
M CostellR Fässler

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 carti...Continue Reading

  • References38
  • Citations321

References

  • References38
  • Citations321

Citations

Mentioned in this Paper

Arsa
Chondrodysplasia
Hspg2
Iniencephaly
Hereditary Multiple Exostoses
Extravasation
Chondrodysplasia Punctata
Fibrillar Collagen
Brain
Extracellular Matrix

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