The idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium.
The idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium.
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DOI：
10.1371/journal.pone.0058658
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发表时间：
2013
期刊：
影响因子：
3.7
通讯作者：
Reynolds SD
中科院分区：
文献类型：
--
作者：
Seibold MA;Smith RW;Urbanek C;Groshong SD;Cosgrove GP;Brown KK;Schwarz MI;Schwartz DA;Reynolds SD
我们之前发现MUC5B基因启动子变异是散发性和家族性特发性肺纤维化/通常间质性肺炎（IPF/UIP）的风险等位基因。该等位基因与未受影响受试者肺组织中MUC5B基因表达的增加密切相关。尽管这种气道上皮标志物与疾病密切相关，但对黏液蛋白表达结构或气道参与IPF/UIP知之甚少。免疫荧光根据MUC5B和MUC5AC的表达对粘液细胞进行分型，并在对照组和IPF/UIP受试者的组织切片中鉴定纤毛、基底和肺泡II型（ATII）细胞。对远端气道（Control和IPF/UIP）和蜂窝囊肿（HC）的染色模式进行量化。在大多数对照组远端气道中检测到muc5b表达细胞（EC）。MUC5AC-EC在一半的气道中被鉴定出来，并且仅在含有MUC5B-EC的气道中被鉴定出来。IPF/UIP患者MUC5B+和MUC5AC+远端气道频率增加。MUC5B-EC是HC上皮中占优势的粘液细胞类型。对照组和IPF/UIP受试者以及HC的远端气道上皮由基底细胞和纤毛细胞填充。大多数蜂窝区与ATII增生区明显不同。在绝大多数HC中检测不到ATII细胞。远端气道含有假层状粘膜纤毛上皮，由主要表达MUC5B的基底上皮细胞和粘液细胞定义。这些数据表明HC来源于远端气道。
We previously identified a MUC5B gene promoter-variant that is a risk allele for sporadic and familial Idiopathic Pulmonary Fibrosis/Usual Interstitial Pneumonia (IPF/UIP). This allele was strongly associated with increased MUC5B gene expression in lung tissue from unaffected subjects. Despite the strong association of this airway epithelial marker with disease, little is known of mucin expressing structures or of airway involvement in IPF/UIP. Immunofluorescence was used to subtype mucus cells according to MUC5B and MUC5AC expression and to identify ciliated, basal, and alveolar type II (ATII) cells in tissue sections from control and IPF/UIP subjects. Staining patterns were quantified for distal airways (Control and IPF/UIP) and in honeycomb cysts (HC). MUC5B-expressing cells (EC) were detected in the majority of control distal airways. MUC5AC-EC were identified in half of these airways and only in airways that contained MUC5B-EC. The frequency of MUC5B+ and MUC5AC+ distal airways was increased in IPF/UIP subjects. MUC5B-EC were the dominant mucus cell type in the HC epithelium. The distal airway epithelium from control and IPF/UIP subjects and HC was populated by basal and ciliated cells. Most honeycombing regions were distinct from ATII hyperplasic regions. ATII cells were undetectable in the overwhelming majority of HC. The distal airway contains a pseudostratified mucocilary epithelium that is defined by basal epithelial cells and mucus cells that express MUC5B predominantly. These data suggest that the HC is derived from the distal airway.
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影响因子：
5.8
作者：
Chilosi, Marco;Zamo, Alberto;Poletti, Venerino
通讯作者：
Poletti, Venerino
影响因子：
5
作者：
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通讯作者：
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影响因子：
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作者：
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通讯作者：
Finkelstein, JN
DOI：
10.1152/ajplung.2000.278.1.l165
发表时间：
2000-01-01
影响因子：
4.9
作者：
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通讯作者：
Puchelle, E
DOI：
10.1164/rccm.200601-062oc
发表时间：
2007-01-01
影响因子：
24.7
作者：
Yang, Ivana V.;Burch, Lauranell H.;Schwartz, David A.
通讯作者：
Schwartz, David A.