论文降重
免费查重- 钛学术文献服务平台 \
- 学术期刊 \
- 综合期刊 \
- 其它期刊 \
- 世界呼吸病学杂志期刊 \
Von Hippel-Lindau protein and respiratory diseases
Von Hippel-Lindau protein and respiratory diseases
基本信息来源于合作网站，原文需代理用户跳转至来源网站获取
摘要：
Von Hippel-Lindau protein(pVHL)was first identified as a tumor suppressor gene as mutations in the VHL gene predispose individuals to systemic benign or malignant tumors and cysts in many organs,including renal cell carcinoma of the clear-cell type and hemangioblastoma.Although pVHL is best known to act as a component of ubiquitin protein ligase for the proteasomal degradation of hypoxia inducible factor(HIF)-α,pVHL also interacts with extracellular matrix proteins and cytoskeleton,regulating extracellular matrix assembly,cell signaling,and many other cellular functions.Recent studies suggest that pVHL contributes to many lung diseases,including pulmonary arterial hypertension,lung cancer,pulmonary fibrosis,and acute respiratory distress syndrome.Mutation or loss of function of pVHL activates HIF and induced expression of vascular endothelial growth factor,endothelin-1,and FoxM1,leading to pulmonary arterial hypertension.Loss of pVHL in lung cancer cells promotes epithelial-mesenchymal transition and cancer migration and invasion while decreasing lung cancer cell proliferation and colonization.In patients of idiopathic pulmonary fibrosis,elevated expression of pVHL induces expression of fibronectin/integrinα5β1/focal adhesion kinase signaling,resulting in fibroproliferation and fibrosis.In alveolar epithelial cells,pVHL mediates Na,K-ATPase degradation in an HIF independent pathway,causing decreased edema clearance during hypoxia.These studies suggest that pVHL plays key roles in the pathogenesis of many lung diseases,and further investigations are warranted to elucidate the underlying molecular mechanisms.
推荐文章
Von Hippel-Lindau病肾癌的临床特征分析
Von Hippel-Lindau(VHL)病
肾细胞癌
Von Hippel-Lindau综合征胰腺病变诊治进展
VonHippel-Lindau(VHL)综合征
胰腺病变
诊治进展
囊性病变
神经内分泌肿瘤
Von Hippel-Lindau综合征2例报告及文献复习
Von Hippel-Lindau综合征
囊肿
肿瘤
诊断
治疗
Von Hippel-Lindau综合征的CT及MRI表现研究
肿瘤
磁共振成像
囊肿
Von Hippel-Lindau综合征
CT
内容分析
关键词云
关键词热度
相关文献总数
(/次)
(/年)
引文网络
引文网络
二级参考文献 (0)
共引文献 (0)
参考文献 (0)
节点文献
引证文献 (0)
同被引文献 (0)
二级引证文献 (0)
2013(0)
- 参考文献(0)
- 二级参考文献(0)
- 引证文献(0)
- 二级引证文献(0)
研究主题发展历程
节点文献
Von
HIPPEL-LINDAU
PROTEIN
LUNG
cancer
PULMONARY
FIBROSIS
PULMONARY
HYPERTENSION
研究起点
研究来源
研究分支
研究去脉
引文网络交叉学科
相关学者/机构
期刊影响力
世界呼吸病学杂志
主办单位：
百世登出版集团有限公司
出版周期：
不定期
ISSN：
2218-6255
CN：
开本：
出版地：
北京市朝阳区东四环中路62号楼远洋国际中
邮发代号：
创刊时间：
语种：
出版文献量（篇）
66
总下载数（次）
0
总被引数（次）
0
期刊文献
相关文献
推荐文献
