Defective epidermal growth factor gene expression in mice with polycystic kidney disease.

Defective epidermal growth factor gene expression in mice with polycystic kidney disease.
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多囊肾病小鼠表皮生长因子基因表达缺陷。

DOI:
10.1016/0012-1606(90)90192-l
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发表时间:
1990
影响因子:
2.7
通讯作者:
Calvet,JP
Calvet,JP
中科院分区:
生物学3区
文献类型:
--
作者:
Gattone2nd,VH;Andrews,GK;Niu,FW;Chadwick,LJ;Klein,RM;Calvet,JP

文献摘要

被引文献

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C57 BL/6 J-cpk小鼠具有与人类常染色体隐性遗传疾病相似的可遗传形式的多囊肾病。在大约1到3周龄之间,受影响的cpk小鼠在肾单位的集合小管段形成许多大囊肿。本研究采用北方杂交和免疫组织化学方法检测了小鼠肾脏和颌下腺前表皮生长因子原(preproEGF)基因表达的个体发生。有一个虚拟的情况下,肾preproEGF基因的表达在囊性肾超过3周出生后的时期,在此期间,肾preproEGF mRNA和proEGF/EGF蛋白通常达到显着的水平。PreproEGF mRNA在囊性小鼠的唾液腺中表达;然而,这种mRNA不能进一步升高,这表明在颌下腺以及肾脏中preproEGF基因的调节存在异常。由于在出生后早期,当集合管囊肿形成时,肾脏preproEGF表达发生,因此可能是肾脏proEGF或EGF缺乏导致C57 BL/6 J-cpkcystic小鼠集合管囊肿的快速发展和伴随的肾衰竭。
The C57BL/6J-cpkmouse has an inheritable form of polycystic kidney disease similar to the autosomal recessive disorder seen in humans. Between approximately 1 and 3 weeks of age, affectedcpkmice develop numerous large cysts in the collecting tubule segment of kidney nephrons. The present study examined the ontogeny of renal and submandibular gland prepro-epidermal growth factor (preproEGF) gene expression in thecpkmouse using Northern blot hybridization and immunohistochemistry. There was a virtual absence of renal preproEGF gene expression in cystic kidneys over the 3-week postnatal period, during which time renal preproEGF mRNA and proEGF/EGF protein normally reach significant levels. PreproEGF mRNA was expressed in salivary glands of cystic mice; however, this mRNA could not be further elevated with testosterone suggesting that there are abnormalities in the regulation of the preproEGF gene in the submandibular gland, as well as in the kidney. Since renal preproEGF expression during the early postnatal period occurs when collecting duct cysts form, it is possible that a deficiency in renal proEGF or EGF contributes to the rapid development of collecting duct cysts and the concomitant renal failure in the C57BL/6J-cpkcystic mouse.