Ty- Jour T1-成人肺肺泡蛋白质中的表面活性剂蛋白JF-欧洲呼吸杂志JO -EUR RESSIR J SP -426 LP -426 LP -435 do -10.1183/09031936.04.000764403 VL -24Birzele，J。Au -Ochs，M.Au -Guttentag，S.H。Au -Schoch，O.D。Au -Boehler，A。Au -Beers，M.F。Au -Müller，K.M。Au -Hawgood，S。Au -Johnen，G。Y1-2004/09/01 Ur -http：//www.qdcxjkg.com/content/content/24/3/426.abstract n2-肺肺泡蛋白质（PAP）是一个在组织学特征的罕见疾病以细粒嗜酸性粒细胞和周期性酸 - chiff阳性材料的肺泡内积累的特征。在一项回顾性研究中，通过免疫组织化学和蛋白质印迹分析了成年患者的肺泡内积累材料的组成。在患有PAP的患者中，目前的作者发现表面活性剂蛋白（SP）-A的肺泡内积累，Sp-B，Sp-B的前体，单，单，DI-和低聚的SP-C形式，如以及sp-d。 Only in one patient was a precursor of SP-C detected. By means of immuno-electron microscopy, the current authors identified not only transport vesicles labelled for precursors of SP-B and SP-C, but also transport vesicles containing either precursors of SP-B or SP-C in type-II pneumocytes in normal human lungs. It is concluded that pulmonary alveolar proteinosis in adults is characterised by an intra-alveolar accumulation of surfactant protein A, precursors of surfactant protein B, and surfactant proteins B, C and D. The current data provide evidence that not only an impairment of surfactant clearance by alveolar macrophages, but also an abnormal secretion of transport vesicles containing precursors of surfactant protein B (but not surfactant protein C) and an insufficient palmitoylation of surfactant protein C, which may lead to the formation of di- and oligomeric surfactant protein C forms, play a role in the pathogenesis of pulmonary alveolar proteinosis. This study was partially supported by NIH P01-19737 (M.F. Beers) and NIH HL-59959 (S.H. Guttentag). ER -