Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice
作者： J. C. Clark , S. E. Wert , C. J. Bachurski , M. T. Stahlman , B. R. Stripp
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摘要: Surfactant protein B (SP-B) is an 8.7-kDa, hydrophobic that enhances the spreading and stability of surfactant phospholipids in alveolus. To further assess role SP-B lung function, gene was disrupted by homologous recombination murine mouse embryonic stem cells. Mice with a single mutated allele (+/-) were unaffected, whereas homozygous -/- offspring died respiratory failure immediately after birth. Lungs mice developed normally but remained atelectatic spite postnatal efforts. mRNA undetectable tubular myelin figures lacking mice. Type II cells contained no fully formed lamellar bodies. While abundance SP-A SP-C mRNAs not altered, aberrant form pro-SP-C, 8.5 kDa, detected, processed peptide markedly decreased homogenates Ablation disrupts routing, storage, function proteins, causing at
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