Advanced

PDGF-A signaling is a critical event in lung alveolar myofibroblast development and alveogenesis.

Ehrencrona, Hans LU ; Willetts, Karen; Pekny, Milos; Levéen, Per; Lindahl, Per; Hedstrand, Håkan; Pekna, Marcela; Hellström, Mats; Gebre-Medhin, Samuel and Schalling, Martin, et al. (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:
author
, et al. (More)
(Less)
publishing date
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
2013-10-21 10:51:04
date last changed
2017-08-13 04:37:21
@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},
  keyword      = {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.},
  volume       = {85},
  year         = {1996},
}