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首页> 外文期刊>American Journal of Physiology >Altered lung surfactant system in a Rab38-deficient rat model of Hermansky-Pudlak syndrome.
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Altered lung surfactant system in a Rab38-deficient rat model of Hermansky-Pudlak syndrome.

机译：Hermansky-Pudlak综合征的Rab38缺陷大鼠模型中肺表面活性物质系统的改变。

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摘要

Several Long-Evans rat substrains carrying the phenotype of oculocutaneous albinism and bleeding diathesis are a rat model of Hermansky-Pudlak syndrome (HPS). The mutation responsible for the phenotype (Ruby) was identified as a point mutation in the initiation codon of Rab38 small GTPase that regulates intracellular vesicle transport. As patients with HPS often develop life-limiting interstitial pneumonia accompanied by abnormal morphology of alveolar type II cells, we investigated lung surfactant system in Long-Evans Cinnamon rats, one strain of the Ruby rats. The lungs showed conspicuous morphology of type II cells containing markedly enlarged lamellar bodies. Surfactant phosphatidylcholine and surfactant protein B were increased in lung tissues and lamellar bodies but not in alveolar lumen. Expression levels of mRNA for surfactant proteins A, B, C, and D were not altered. Isolated type II cells showed aberrant secretory pattern of newly synthesized [(3)H]phosphatidylcholine, i.e., decreased basal secretion and remarkably amplified agonist-induced secretion. [(3)H]phosphatidylcholine synthesis and uptake by type II cells were not altered. Thus Rab38-deficient type II cells appear to carry abnormality in lung surfactant secretion but not in synthesis or uptake. These results suggest that aberrant lung surfactant secretion may be involved in the pathogenesis of interstitial pneumonia in HPS.
机译：几种带有表皮白化病表型和出血性素质的Long-Evans大鼠亚菌株是Hermansky-Pudlak综合征（HPS）的大鼠模型。负责表型的突变（Ruby）被确定为Rab38小GTP酶的起始密码子中的点突变，该突变调节细胞内囊泡运输。由于患有HPS的患者经常会出现限制生命的间质性肺炎，并伴有II型肺泡细胞形态异常，因此我们对Long-Evans肉桂大鼠（一种Ruby大鼠）的肺表面活性剂系统进行了研究。肺表现出明显的II型细胞形态，其中包含明显扩大的层状体。表面活性剂磷脂酰胆碱和表面活性剂蛋白B在肺组织和片状体中增加，但在肺泡腔中没有增加。表面活性剂蛋白A，B，C和D的mRNA表达水平未改变。分离的II型细胞显示出新合成的[（3）H]磷脂酰胆碱的异常分泌模式，即基础分泌减少，激动剂诱导的分泌明显增加。 [（3）H]磷脂酰胆碱的合成和II型细胞的吸收没有改变。因此，缺乏Rab38的II型细胞似乎在肺表面活性物质分泌方面异常，但在合成或摄取方面却没有异常。这些结果表明，肺表面活性物质的异常分泌可能与HPS间质性肺炎的发病机制有关。

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