Pulmonary Hypoplasia in the myogenin Null Mouse Embryo
| Autor: | Sash T. Cavin, Eric N. Olson, Frank W. Booth, Timothy J. McDonnell, Maria C. Marin, Brian S. Tseng, Ian J. Butler |
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| Rok vydání: | 2000 |
| Předmět: |
Pulmonary and Respiratory Medicine
Heterozygote Pathology medicine.medical_specialty Pulmonary Surfactant-Associated Proteins Proteolipids Clinical Biochemistry Apoptosis Biology Immunoenzyme Techniques Mice Pulmonary hypoplasia Immunolabeling Proto-Oncogene Proteins In Situ Nick-End Labeling medicine Animals Kyphosis RNA Messenger Molecular Biology Myogenin bcl-2-Associated X Protein Cyanosis Lung Respiration Homozygote Gene Expression Regulation Developmental Skeletal muscle Pulmonary Surfactants Embryo Organ Size Cell Biology medicine.disease Mice Mutant Strains Respiratory Muscles Pulmonary Alveoli medicine.anatomical_structure Proto-Oncogene Proteins c-bcl-2 In utero embryonic structures Lung cell differentiation Cell Division |
| Zdroj: | American Journal of Respiratory Cell and Molecular Biology. 22:304-315 |
| ISSN: | 1535-4989 1044-1549 |
| DOI: | 10.1165/ajrcmb.22.3.3708 |
| Popis: | Although fetal breathing movements are required for normal lung development, there is uncertainty concerning the specific effect of absent fetal breathing movements on pulmonary cell maturation. We set out to evaluate pulmonary development in a genetically defined mouse model, the myogenin null mouse, in which there is a lack of normal skeletal muscle fibers and thus skeletal muscle movements are absent in utero. Significant decreases were observed in lung:body weight ratio and lung total DNA at embryonic days (E)14, E17, and E20. Reverse transcriptase/polymerase chain reaction, in situ immunofluorescence, and electron microscopy revealed early lung cell differentiation in both null and wild-type lungs as early as E14. However at E14, myogenin null lungs had decreased 5'-bromo-2-deoxyuridine incorporation compared with that of wild-type littermates, whereas at E17 and E20, increased Bax immunolabeling and terminal deoxyribonucleotidyl transferase-mediated dUTP-biotin nick-end labeling staining were detected in the myogenin null mice but not in the wild-type littermates. These observations highlight the importance of skeletal muscle contractile activity in utero for normal lung organogenesis. Null mice lacking the muscle-specific transcription factor myogenin exhibit a secondary effect on lung development such that decreased lung cell proliferation and increased programmed cell death are associated with lung hypoplasia. |
| Databáze: | OpenAIRE |
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