| First Author | Baguma-Nibasheka M | Year | 2008 |
| Journal | Dev Dyn | Volume | 237 |
| Issue | 2 | Pages | 485-93 |
| PubMed ID | 18213577 | Mgi Jnum | J:130165 |
| Mgi Id | MGI:3771125 | Doi | 10.1002/dvdy.21433 |
| Citation | Baguma-Nibasheka M, et al. (2008) Pulmonary hypoplasia in the connective tissue growth factor (Ctgf) null mouse. Dev Dyn 237(2):485-93 |
| abstractText | Connective tissue growth factor (CTGF) is a mediator of growth factor activity, and Ctgf knockouts die at birth from respiratory failure due to skeletal dysplasia. Previous microarray analysis revealed Ctgf down-regulation in the hypoplastic lungs of amyogenic mouse embryos. This study, therefore, examined pulmonary development in Ctgf-/- mouse fetuses to investigate if respiration could also have been impaired by lung abnormalities. The Ctgf-/- lungs were hypoplastic, with reduced cell proliferation and increased apoptosis. PDGF-B, its receptor and IGF-I, were markedly attenuated and the TTF-1 gradient lost. Type II pneumocyte differentiation was perturbed, the cells depicting excessive glycogen retention and diminished lamellar body and nuclear size, though able to synthesize surfactant-associated protein. However, type I pneumocyte differentiation was not affected by Ctgf deletion. Our findings indicate that the absence of Ctgf and/or its protein product, CTGF, may induce pulmonary hypoplasia by both disrupting basic lung developmental processes and restricting thoracic expansion. Developmental Dynamics 237:485-493, 2008. (c) 2008 Wiley-Liss, Inc. |
