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表面活性蛋白B基因的靶向破坏会扰乱表面活性剂的稳态，导致新生小鼠呼吸衰竭。

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

作者信息

Clark J C, Wert S E, Bachurski C J, Stahlman M T, Stripp B R, Weaver T E, Whitsett J A

机构信息

Division of Pulmonary Biology, Children's Hospital Medical Center, Cincinnati, OH 45229-3039, USA.

出版信息

Proc Natl Acad Sci U S A. 1995 Aug 15;92(17):7794-8. doi: 10.1073/pnas.92.17.7794.

DOI:10.1073/pnas.92.17.7794
PMID:7644495
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC41232/
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.

摘要

表面活性蛋白B（SP-B）是一种8.7 kDa的疏水蛋白，可增强表面活性磷脂在肺泡中的扩散和稳定性。为了进一步评估SP-B在肺功能中的作用，通过同源重组在小鼠胚胎干细胞中破坏了SP-B基因。携带单个突变SP-B等位基因（+/-）的小鼠未受影响，而纯合的SP-B -/-后代在出生后立即死于呼吸衰竭。SP-B -/-小鼠的肺正常发育，但尽管出生后进行了呼吸努力，仍保持肺不张。在SP-B -/-小鼠中检测不到SP-B蛋白和mRNA，也没有管状髓磷脂图像。SP-B -/-小鼠的II型细胞中没有完全形成的板层小体。虽然SP-A和SP-C mRNA的丰度没有改变，但检测到一种异常形式的前SP-C，8.5 kDa，并且在SP-B -/-小鼠的肺匀浆中完全加工的SP-C肽明显减少。SP-B基因的缺失破坏了表面活性磷脂和蛋白质的转运、储存和功能，导致出生时呼吸衰竭。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6da9/41232/b7a42b3397c9/pnas01495-0200-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6da9/41232/c6b4d84fbbf7/pnas01495-0198-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6da9/41232/e8018ef0a7db/pnas01495-0199-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6da9/41232/092981f63c10/pnas01495-0199-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6da9/41232/feb19a983772/pnas01495-0199-c.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6da9/41232/b7a42b3397c9/pnas01495-0200-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6da9/41232/c6b4d84fbbf7/pnas01495-0198-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6da9/41232/e8018ef0a7db/pnas01495-0199-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6da9/41232/092981f63c10/pnas01495-0199-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6da9/41232/feb19a983772/pnas01495-0199-c.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6da9/41232/b7a42b3397c9/pnas01495-0200-a.jpg

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