| Autor: |
Clark JC; Division of Neonatology, Children's Hospital Medical Center, Cincinnati, Ohio 45229-3039, USA., Weaver TE, Iwamoto HS, Ikegami M, Jobe AH, Hull WM, Whitsett JA |
| Jazyk: |
angličtina |
| Zdroj: |
American journal of respiratory cell and molecular biology [Am J Respir Cell Mol Biol] 1997 Jan; Vol. 16 (1), pp. 46-52. |
| DOI: |
10.1165/ajrcmb.16.1.8998078 |
| Abstrakt: |
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: |
MEDLINE |
| Externí odkaz: |
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