Decreased lung compliance and air trapping in heterozygous SP-B-deficient mice
| Autor: | Harriet S. Iwamoto, William M. Hull, Jean C. Clark, Timothy E. Weaver, Jeffrey A. Whitsett, Alan H. Jobe, Machiko Ikegami |
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| Rok vydání: | 1997 |
| Předmět: |
Male
Pulmonary and Respiratory Medicine Genetically modified mouse Heterozygote medicine.medical_specialty Pathology Offspring Proteolipids Clinical Biochemistry Mice Transgenic Pulmonary compliance Biology Pulmonary function testing Mice Internal medicine medicine Extracellular Animals RNA Messenger Lung Lung Compliance Molecular Biology Phospholipids Gene targeting Pulmonary Surfactants Heterozygote advantage Cell Biology Pulmonary Alveoli medicine.anatomical_structure Endocrinology Gene Targeting Respiratory Mechanics Female Lung Volume Measurements |
| Zdroj: | American Journal of Respiratory Cell and Molecular Biology. 16:46-52 |
| ISSN: | 1535-4989 1044-1549 |
| DOI: | 10.1165/ajrcmb.16.1.8998078 |
| Popis: | Genetic ablation of the murine SP-B gene in transgenic mice caused lethal perinatal respiratory distress in homozygous offspring, whereas heterozygous SP-B (+/-) mice survived postnatally. In adult SP-B(+/-) mice, surfactant protein B mRNA and the alveolar lavage SP-B protein were reduced by 50% compared with wild-type littermates, consistent with the inactivation of a single SP-B allele. Expression of SP-A, SP-C, and SP-D proteins was not affected in SP-B(+/-) mice. Heterozygous SP-B(+/-) mice reached maturity in numbers expected by Mendelian inheritance of a recessive gene. Lung morphology and both intracellular and extracellular phospholipid pool size and composition were unaltered in the SP-B(+/-) mice. Despite normal survival, pulmonary function studies demonstrated a consistent decrease in lung compliance in SP-B(+/-) mice. Abnormalities of inflation/deflation curves demonstrated airway collapse at low deflation pressures. Residual volumes were increased in the SP-B(+/-) mice. In summary, SP-B mRNA and SP-B protein were reduced by 50% in SP-B(+/-) mice, resulting in abnormalities of lung compliance and air trapping, suggesting a potential susceptibility to pulmonary dysfunction associated with SP-B deficiency. |
| Databáze: | OpenAIRE |
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