PDGF-A signaling is a critical event in lung alveolar myofibroblast development and alveogenesis.
(1996) In Cell 85(6). p.863-873- Abstract
- 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... (More)
- 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. (Less)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/4021999
- author
- publishing date
- 1996
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- Pulmonary Alveoli, Platelet-Derived Growth Factor, Phenotype, Smooth, Muscle, Mutant Strains, Mice, Lung, Gene Targeting, Fibroblasts, Elastin, Genetic, Crosses, Chimera, Cardiomegaly, Actins, Animals, Pulmonary Emphysema, RNA, Messenger, Receptor, Platelet-Derived Growth Factor alpha, Receptors, Signal Transduction
- in
- Cell
- volume
- 85
- issue
- 6
- pages
- 863 - 873
- publisher
- Cell Press
- external identifiers
-
- scopus:15844370315
- ISSN
- 1097-4172
- language
- English
- LU publication?
- no
- id
- 237cf46b-ce3c-4cbc-b05b-8c917de31b1a (old id 4021999)
- alternative location
- http://www.ncbi.nlm.nih.gov/pubmed/8681381
- date added to LUP
- 2016-04-04 08:56:38
- date last changed
- 2022-03-31 00:43:11
@article{237cf46b-ce3c-4cbc-b05b-8c917de31b1a, abstract = {{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.}}, author = {{Ehrencrona, Hans and Willetts, Karen and Pekny, Milos and Levéen, Per and Lindahl, Per and Hedstrand, Håkan and Pekna, Marcela and Hellström, Mats and Gebre-Medhin, Samuel and Schalling, Martin and Nilsson, Mia and Kurland, Siri and Törnell, Jan and Heath, John K and Betsholtz, Christer}}, issn = {{1097-4172}}, keywords = {{Pulmonary Alveoli; Platelet-Derived Growth Factor; Phenotype; Smooth; Muscle; Mutant Strains; Mice; Lung; Gene Targeting; Fibroblasts; Elastin; Genetic; Crosses; Chimera; Cardiomegaly; Actins; Animals; Pulmonary Emphysema; RNA; Messenger; Receptor; Platelet-Derived Growth Factor alpha; Receptors; Signal Transduction}}, language = {{eng}}, number = {{6}}, pages = {{863--873}}, publisher = {{Cell Press}}, series = {{Cell}}, title = {{PDGF-A signaling is a critical event in lung alveolar myofibroblast development and alveogenesis.}}, url = {{http://www.ncbi.nlm.nih.gov/pubmed/8681381}}, volume = {{85}}, year = {{1996}}, }