排序方式： 共有1条查询结果，搜索用时 15 毫秒
1
1.
Polycystic kidney diseases (PKDs) represent a large group of progressive renal disorders characterized by the development
of renal cysts leading to end-stage renal disease. Enormous strides have been made in understanding the pathogenesis of PKDs
and the development of new therapies. Studies of autosomal dominant and recessive polycystic kidney diseases converge on molecular
mechanisms of cystogenesis, including ciliary abnormalities and intracellular calcium dysregulation, ultimately leading to
increased proliferation, apoptosis and dedifferentiation. Here we review the pathobiology of PKD, highlighting recent progress
in elucidating common molecular pathways of cystogenesis. We discuss available models and challenges for therapeutic discovery
as well as summarize the results from preclinical experimental treatments targeting key disease-specific pathways.
Received 8 August 2007; received after revision 19 September 2007; accepted 2 October 2007 相似文献
1