期刊论文

Wu, Guanqing

英文

Experimental Cell Research

0014-4827

2011

317

2

173-187

10.1016/j.yexcr.2010.09.012

National Institutes of Health of the USAUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USA [DK062373, DK71090]; National Cancer Institute Specialized Programs of Research Excellence, NIH, USAUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USA [5P50 CA095103]; National Natural Science Foundation of ChinaNational Natural Science Foundation of China (NSFC) [30672483, 30870501]; Changjiang Scholarship of China; State Key Laboratory of Molecular Oncology of China; NATIONAL CANCER INSTITUTEUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Cancer Institute (NCI) [P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103, P50CA095103] Funding Source: NIH RePORTER; NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASESUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute of Diabetes & Digestive & Kidney Diseases (NIDDK) [R21DK065111, R01DK071090, R01DK062373, R01DK062373, R01DK071090, R01DK062373, R01DK071090, R01DK071090, R01DK062373, R01DK062373, R21DK065111] Funding Source: NIH RePORTER; OFFICE OF THE DIRECTOR, NATIONAL INSTITUTES OF HEALTHUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USA [R21OD012387, R21OD012387, R21OD012387] Funding Source: NIH RePORTER

本校为第一机构

Mutations in the PKHD1 gene result in autosomal recessive polycystic kidney disease (ARPKD) in humans. To determine the molecular mechanism of the cystogenesis in ARPKD, we recently generated a mouse model for ARPKD that carries a targeted mutation in the mouse orthologue of human PKHD1. The homozygous mutant mice display hepatorenal cysts whose phenotypes are similar to those of human ARPKD patients. By littermates of this mouse, we developed two immortalized renal collecting duct cell lines with Pkhd1 and two without. Under nonpermissive culture conditions, the Pkhd1(-/-) renal cells display...