Technique for estimating fetal mouse thoracic volumes through image analysis of histological sections

Background: The mutant chondrodysplasia (cho) is a cartilage-targeting disorder in C57BL mice that results in dwarfing and other malformations stemming from this collagenopathy. Clarke Fraser made the discovery of the mutation accidentally in the early 1960s during the thalidomide tragedy. Methods: For this review we identified key research on cho as since its discovery. Relevant data were compiled to make a comprehensive review that details discoveries associated with the cho mutation, that describes the associated phenotypes and molecular mechanisms, and that provides a discussion surrounding its current clinical relevance. Results: Mechanistically, cho acts by hindering chondrogenesis and endochondral bone formation. The phenotype results from a 1-nt deletion in the gene encoding the alpha 1 chain of type XI collagen. For more than half a century, researchers have studied the pathogenesis of the cho mutation in relation to a variety of mouse models of human birth defects and disease. These studies have resulted in several discoveries linking cho with such human disorders as dwarfism, tracheal stenosis, cleft palate, pulmonary hypoplasia, and osteoarthritis (OA). Conclusion: The study of cho has led to numerous advances in understanding human birth defects, congenital disorders, and adult human disease. The most recent studies have suggested a role for the TGF-Beta, HtrA1, Ddr2, and Mmp-13 pathway in the degradation of articular cartilage and the development of OA in cho/+ mice. We have shown that the anti-hypertension drug Losartan is a TGF-Beta blocker that could be used to treat OA in Stickler syndrome, and thereby rescue the WT phenotype.

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Understanding chondrodysplasia (cho): A comprehensive review of cho as an animal model of birth defects, disorders, and molecular mechanisms

Seegmiller

Foster

Burnham

2019

Birth Defects Research

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Arrested pulmonary alveolar cytodifferentiation and defective surfactant synthesis in mice missing the gene for parathyroid hormone‐related protein

Rubin

Kovacs

Paepe

et al. 2004

Developmental Dynamics

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Pulmonary Hypoplasia in the myogenin Null Mouse Embryo

Tseng

Cavin

Booth

et al. 2000

Am J Respir Cell Mol Biol

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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.

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Technique for estimating fetal mouse thoracic volumes through image analysis of histological sections

Cited by 5 publications

References 14 publications

Understanding chondrodysplasia (cho): A comprehensive review of cho as an animal model of birth defects, disorders, and molecular mechanisms

Understanding chondrodysplasia (cho): A comprehensive review of cho as an animal model of birth defects, disorders, and molecular mechanisms

Arrested pulmonary alveolar cytodifferentiation and defective surfactant synthesis in mice missing the gene for parathyroid hormone‐related protein

Pulmonary Hypoplasia in the myogenin Null Mouse Embryo

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