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Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice.

Open access
Clark, J C, Wert, S E, Bachurski, C J, Stahlman, M T, Stripp, B R, Weaver, T E, Whitsett, J A·1995·Proceedings of the National Academy of Sciences

Abstract

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

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Metadata

Authors
Clark, J. C., Wert, S. E., Bachurski, C. J., Stahlman, M. T., Stripp, B. R., Weaver, T. E., & Whitsett, J. A.
Venue
Proceedings of the National Academy of Sciences
Published
1995-08-15
Volume
92
Issue
17
Pages
7794-7798
ISSN
  • 0027-8424 (ISSN-L)
  • 1091-6490
OpenAlex
W1990402565
Cited by
642
References
0
FWCI
24.60
Type
journal article
Language
en
Open access
green

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CoChat. (2026). Citation check: Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice. https://app.cochat.ai/papers/10.1073/pnas.92.17.7794
The paper
Clark, J. C., Wert, S. E., Bachurski, C. J., Stahlman, M. T., Stripp, B. R., Weaver, T. E., & Whitsett, J. A. (1995). Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice. Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.92.17.7794

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