Developmental expression of a mucinlike glycoprotein (MUCLIN) in pancreas and small intestine of CF mice.
Developmental expression of a mucinlike glycoprotein (MUCLIN) in pancreas and small intestine of CF mice.
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CF 小鼠胰腺和小肠中粘蛋白样糖蛋白 (MUCLIN) 的发育表达。
DOI：
10.1152/ajpgi.1998.275.2.g219
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发表时间：
1998
期刊：
影响因子：
--
通讯作者：
Ziemer,D
中科院分区：
文献类型：
--
作者：
DeLisle,RC;Petitt,M;Isom,KS;Ziemer,D
使用囊性纤维化跨膜传导调节因子（CFTR）敲除小鼠（CF小鼠），将CRP-导管蛋白的两种蛋白产物之一粘蛋白样糖蛋白MUCLIN用于研究囊性纤维化发病过程中硫酸化糖缀合物表达的变化。我们评估了出生后发育过程中胰腺腺泡和小肠隐窝中含有蛋白质或粘液栓的扩张管腔的外观，并定量了MUCLIN蛋白和CRP-导管蛋白mRNA。与正常小鼠相比，CF小鼠的胰腺腺泡腔在出生后16天（P16）扩张，但MUCLIN蛋白在出生后23天开始显著增加，并在成年后一直保持升高。与此类似，与正常小鼠相比，肠隐窝在P16时有CF样粘液栓，但MUCLIN蛋白在P23时首次升高，并在成年期保持升高。在这两个器官中，腔表面的MUCLIN标记随着扩张和蛋白质或粘液堵塞而增加，但在表达上调之前。形态学改变之后，MUCLIN蛋白和CRP-导管蛋白mRNA表达上调。这是第一次直接研究CF发病机制和糖缀合物基因表达的增加。这些数据与CF发病机制一致，从蛋白质分泌动力学的初始改变（管腔MUCLIN和蛋白质/粘液栓增加）发展为糖蛋白/粘蛋白基因表达的上调，预计这会加剧管腔空间的阻塞。
The mucinlike glycoprotein MUCLIN, one of two protein products of theCRP-ductingene, was used to study changes in the expression of sulfated glycoconjugates during the pathogenesis of cystic fibrosis, using the cystic fibrosis transmembrane conductance regulator (CFTR) knockout mouse (CF mouse). We assessed the appearance of dilated lumina containing protein or mucus plugs in pancreatic acini and crypts of the small intestine and quantified MUCLIN protein andCRP-ductinmRNA during postnatal development. In CF mice, the pancreatic acinar lumen was dilated by postnatalday 16(P16), but MUCLIN protein was first significantly increased byP23and remained elevated through adulthood compared with normal mice. Similarly, intestinal crypts had CF-like mucus plugs byP16, but MUCLIN protein was first elevated byP23and remained elevated through adulthood compared with normal mice. In both organs, MUCLIN labeling of the luminal surface was increased concomitantly with dilation and protein or mucus plugging but before upregulation of expression. The morphological changes were then followed by upregulation of MUCLIN protein andCRP-ductinmRNA expression. This is the first direct study of CF pathogenesis and the resultant increase in glycoconjugate gene expression. The data are consistent with CF pathogenesis progressing from an initial alteration in protein secretory dynamics (increased luminal MUCLIN and protein/mucus plugs) to an upregulation of glycoprotein/mucin gene expression, which is expected to exacerbate obstruction of the luminal spaces.
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影响因子：
30.8
作者：
Rozmahel, R;Wilschanski, M;Tsui, LC
通讯作者：
Tsui, LC
囊性纤维化的小鼠模型。
DOI：
10.1164/ajrccm/151.3_pt_2.s59
发表时间：
1995
期刊：
American journal of respiratory and critical care medicine.
影响因子：
--
作者：
Snouwaert,JN;Brigman,KK;Latour,AM;Iraj,E;Schwab,U;Gilmour,MI;Koller,BH
通讯作者：
Koller,BH
囊性纤维化跨膜电导调节剂的鼠 cDNA 的分子克隆和序列分析。
影响因子：
4.4
作者：
Yorifuji,T;Lemna,WK;Ballard,CF;Rosenbloom,CL;Rozmahel,R;Plavsic,N;Tsui,LC;Beaudet,AL
通讯作者：
Beaudet,AL
完整膜蛋白的定量斑点印迹免疫分析：含有顶端和基底外侧结构域的胰腺质膜的制备。
影响因子：
2.9
作者：
DeLisle,RC
通讯作者：
DeLisle,RC
MUCLIN 在囊性纤维化跨膜电导调节因子敲除小鼠中的表达。
影响因子：
29.4
作者：
DeLisle,RC;Petitt,M;Huff,J;Isom,KS;Agbas,A
通讯作者：
Agbas,A