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PDGF-A signaling is a critical event in lung alveolar myofibroblast development and alveogenesis.
Uppsala University, Medicinska vetenskapsområdet, Faculty of Medicine, Department of Genetics and Pathology.
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1996 (English)In: Cell, ISSN 0092-8674, Vol. 85, no 6, 863-73 p.Article in journal (Refereed) Published
Abstract [en]

A mouse platelet-derived growth factor A chain (PDGF-A) null allele is shown to be homozygous lethal, with two distinct restriction points, one prenatally before E10 and one postnatally. Postnatally surviving PDGF-A-deficient mice develop lung emphysema secondary to the failure of alveolar septation. This is apparently caused by the loss of alveolar myofibroblasts and associated elastin fiber deposits. PDGF alpha receptor-positive cells in the lung having the location of putative alveolar myofibroblast progenitors were specifically absent in PDGF-A null mutants. We conclude that PDGF-A is crucial for alveolar myofibroblast ontogeny. We have previously shown that PDGF-B is required in the ontogeny of kidney mesangial cells. The PDGFs therefore appear to regulate the generation of specific populations of myofibroblasts during mammalian development. The two PDGF null phenotypes also reveal analogous morphogenetic functions for myofibroblast-type cells in lung and kidney organogenesis.

Place, publisher, year, edition, pages
1996. Vol. 85, no 6, 863-73 p.
Keyword [en]
Actins/analysis, Animals, Cardiomegaly/pathology, Chimera, Crosses; Genetic, Elastin/analysis, Fibroblasts/cytology/pathology, Gene Targeting, Lung/embryology/ultrastructure, Mice, Mice; Mutant Strains, Muscle; Smooth/chemistry/cytology, Phenotype, Platelet-Derived Growth Factor/deficiency/genetics/*physiology, Pulmonary Alveoli/chemistry/cytology/*growth & development/pathology, Pulmonary Emphysema/*pathology, RNA; Messenger/analysis, Receptor; Platelet-Derived Growth Factor alpha, Receptors; Platelet-Derived Growth Factor/analysis, Research Support; Non-U.S. Gov't, Signal Transduction/physiology
URN: urn:nbn:se:uu:diva-73162PubMedID: 8681381OAI: oai:DiVA.org:uu-73162DiVA: diva2:101073
Available from: 2005-06-01 Created: 2005-06-01 Last updated: 2011-01-15

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Boström, HansHedstrand, Håkan
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