BPIFB1 (LPLUNC1) is upregulated in cystic fibrosis lung disease.
BPIFB1 (LPLUNC1) is upregulated in cystic fibrosis lung disease.
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BPIFB1 (LPLUNC1) 在囊性纤维化肺病中表达上调。
DOI：
10.1007/s00418-012-0990-8
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发表时间：
2012-11
影响因子：
2.3
通讯作者：
Bingle, Colin D.
中科院分区：
文献类型：
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作者：
Bingle, Lynne;Wilson, Kirsty;Musa, Maslinda;Araujo, Bianca;Rassl, Doris;Wallace, William A.;LeClair, Elizabeth E.;Mauad, Thais;Zhou, Zhe;Mall, Marcus A.;Bingle, Colin D.
尽管对PLUNC(最近被重新命名为BPI折叠，BPIF)分泌蛋白家族的生物学了解甚少，但基于多个阵列的研究表明，其中一些在肺部疾病中存在差异表达。我们检测了典型的包含两个结构域的家族成员BPIFB1(LPLunc1)在CF患者的肺和CF肺部疾病小鼠模型中的表达。BPIFB1与BPIFA1、MUC5AC、CD68和NE一起定位于CF肺组织，并与正常肺组织和细菌性肺炎组织直接比较。我们产生了针对小鼠BPIF蛋白的新抗体，用于在ENaC转基因(ENaC-TG)小鼠上进行类似的研究，ENaC-TG小鼠是一种CF样肺部疾病的模型。慢性肺炎组小气道上皮杯状细胞表达BPIFB1，肺泡区未见表达。BPIFA1和BPIFB1在病变肺中没有共定位。在ENaC-TG小鼠中，在呼吸道和腔内容物中都有很强的蛋白染色。这对BPIFB1来说是最有意义的，并在出生后2周内被注意到。这两种蛋白存在于不同的上皮细胞中。BPIFB1在ENaC-TG小鼠的BAL中很容易检测到，而在野生型小鼠中则不存在。在人类和小鼠中，BPIF蛋白表达的改变与CF肺部疾病有关。目前尚不清楚这种蛋白质产量的增加是否在疾病的发病机制中发挥了作用，这种增加是由病变上皮细胞的表型变化引起的。本文的在线版本(doi：10.1007/s00418-0120990-8)包含补充材料，授权用户可以使用。
Although the biology the PLUNC (recently renamed BPI fold, BPIF) family of secreted proteins is poorly understood, multiple array based studies have suggested that some are differentially expressed in lung diseases. We have examined the expression of BPIFB1 (LPLUNC1), the prototypic two-domain containing family member, in lungs from CF patients and in mouse models of CF lung disease. BPIFB1 was localized in CF lung samples along with BPIFA1, MUC5AC, CD68 and NE and directly compared to histologically normal lung tissues and that of bacterial pneumonia. We generated novel antibodies to mouse BPIF proteins to conduct similar studies on ENaC transgenic (ENaC-Tg) mice, a model for CF-like lung disease. Small airways in CF demonstrated marked epithelial staining of BPIFB1 in goblet cells but staining was absent from alveolar regions. BPIFA1 and BPIFB1 were not co-localised in the diseased lungs. In ENaC-Tg mice there was strong staining of both proteins in the airways and luminal contents. This was most marked for BPIFB1 and was noted within 2 weeks of birth. The two proteins were present in distinct cells within epithelium. BPIFB1 was readily detected in BAL from ENaC-Tg mice but was absent from wild-type mice. Alterations in the expression of BPIF proteins is associated with CF lung disease in humans and mice. It is unclear if this elevation of protein production, which results from phenotypic alteration of the cells within the diseased epithelium, plays a role in the pathogenesis of the disease. The online version of this article (doi:10.1007/s00418-012-0990-8) contains supplementary material, which is available to authorized users.
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DOI：
10.1152/ajplung.90388.2008
发表时间：
2009-01
影响因子：
4.9
作者：
Kesimer, Mehmet;Kirkham, Sara;Pickles, Raymond J.;Henderson, Ashley G.;Alexis, Neil E.;DeMaria, Genevieve;Knight, David;Thornton, David J.;Sheehan, John K.
通讯作者：
Sheehan, John K.
影响因子：
7.3
作者：
Bingle, L;Cross, SS;Bingle, CD
通讯作者：
Bingle, CD
影响因子：
4.3
作者：
Kraft-Terry, Stephanie D.;Gendelman, Howard E.
通讯作者：
Gendelman, Howard E.
影响因子：
3.4
作者：
Guo, YR;Ma, SF;Garcia, JGN
通讯作者：
Garcia, JGN
DOI：
10.1016/s0167-4781(00)00196-2
发表时间：
2000-10-02
期刊：
BIOCHIMICA ET BIOPHYSICA ACTA-GENE STRUCTURE AND EXPRESSION
影响因子：
--
作者：
Bingle, CD;Bingle, L
通讯作者：
Bingle, L