[HTML][HTML] Development and characterization of a cholangiocyte cell line from the PCK rat, an animal model of Autosomal Recessive Polycystic Kidney Disease
MA Muff, TV Masyuk, AJ Stroope, BQ Huang… - Laboratory …, 2006 - Elsevier
In the PCK rat, a rodent model of Autosomal Recessive Polycystic Kidney Disease (ARPKD),
a spontaneous splicing mutation of Pkhd1 initiates hepatic cyst development. Cystic
cholangiocytes possess short and malformed cilia that do not express fibrocystin, the Pkhd1
protein. During the disease course, cysts continue to grow; however, the mechanisms
underlying cyst progression are unclear due in part to the lack of suitable cell lines to study
cystogenesis. Here, we describe the development of a PCK-derived cholangiocyte cell line …
a spontaneous splicing mutation of Pkhd1 initiates hepatic cyst development. Cystic
cholangiocytes possess short and malformed cilia that do not express fibrocystin, the Pkhd1
protein. During the disease course, cysts continue to grow; however, the mechanisms
underlying cyst progression are unclear due in part to the lack of suitable cell lines to study
cystogenesis. Here, we describe the development of a PCK-derived cholangiocyte cell line …
