Perlecan Maintains the Integrity of Cartilage and Some Basement Membranes
Mercedes Costell,Erika Gustafsson,Attila Aszódi,Matthias Mörgelin,Wilhelm Bloch,Ernst B. Hunziker,Klaus Addicks,Rupert Timpl,Reinhard Fässler +8 more
TLDR
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 cartilageextracllular matrix from degradation.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.read more
Citations
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Basement membranes: structure, assembly and role in tumour angiogenesis
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Collagen IV is essential for basement membrane stability but dispensable for initiation of its assembly during early development
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Composition and structure of articular cartilage: a template for tissue repair.
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