Retinoblastoma (Rb) is the most common malignant'cancer of eye.So-Rb_(50) is the first Rb cell line established in China in 1988.It has passed to the 387th passage now.We collected cells of the 327th passage of SO...Retinoblastoma (Rb) is the most common malignant'cancer of eye.So-Rb_(50) is the first Rb cell line established in China in 1988.It has passed to the 387th passage now.We collected cells of the 327th passage of SO-Rb_(50),purified its genomic DNA and detected it with Rb and c-myc cDNA probes respectively(normal human white blood cells DNA was the control).We found the Rb gene was deleted while c-myc gene was amplified three times.This provides a basis for further study of the regulation of tumor development and tumor reversal with this cell line in vitro.Eye Science 1993;9:34-37.展开更多
Primary biliary cholangitis (PBC) is an autoimmune cholestatic liver disease that progresses to fibrosis and cirrhosis, resulting from the gradual destruction of intrahepatic bile ducts. Exploring genetic variants ass...Primary biliary cholangitis (PBC) is an autoimmune cholestatic liver disease that progresses to fibrosis and cirrhosis, resulting from the gradual destruction of intrahepatic bile ducts. Exploring genetic variants associated with PBC is essential to understand the pathogenesis of PBC. Here we identify a zebrafish balloon dog (blg) mutant with intrahepatic bile duct branching defects, exhibiting several key pathological PBC-like features, including immunodominant autoantigen PDC-E2 production, cholangiocyte apoptosis, immune cell infiltration, inflammatory activation, and liver fibrosis. blg encodes the protein phosphatase 1 regulatory subunit 21 (Ppp1r21), which is enriched in the liver and its peripheral tissues and plays a vital role in the early intrahepatic bile duct formation stage. Further studies show an excessive activation of the PI3K/AKT/mTOR pathway in the hepatic tissues in the mutant, while treatment with the pathway inhibitor LY294002 and rapamycin partially rescues intrahepatic bile duct branching defects and alleviates the PBC-like symptoms. These findings implicate the potential role of the Ppp1r21-mediated PI3K/AKT/mTOR pathway in the pathophysiology of PBC.展开更多
基金The project was supported by National Natural Science Foundation of China(NAFC)
文摘Retinoblastoma (Rb) is the most common malignant'cancer of eye.So-Rb_(50) is the first Rb cell line established in China in 1988.It has passed to the 387th passage now.We collected cells of the 327th passage of SO-Rb_(50),purified its genomic DNA and detected it with Rb and c-myc cDNA probes respectively(normal human white blood cells DNA was the control).We found the Rb gene was deleted while c-myc gene was amplified three times.This provides a basis for further study of the regulation of tumor development and tumor reversal with this cell line in vitro.Eye Science 1993;9:34-37.
基金This work was supported by the National Natural Science Foundation of China(32270859 and 32192400)the National Key R&D Program of China(2021YFA0805000).
文摘Primary biliary cholangitis (PBC) is an autoimmune cholestatic liver disease that progresses to fibrosis and cirrhosis, resulting from the gradual destruction of intrahepatic bile ducts. Exploring genetic variants associated with PBC is essential to understand the pathogenesis of PBC. Here we identify a zebrafish balloon dog (blg) mutant with intrahepatic bile duct branching defects, exhibiting several key pathological PBC-like features, including immunodominant autoantigen PDC-E2 production, cholangiocyte apoptosis, immune cell infiltration, inflammatory activation, and liver fibrosis. blg encodes the protein phosphatase 1 regulatory subunit 21 (Ppp1r21), which is enriched in the liver and its peripheral tissues and plays a vital role in the early intrahepatic bile duct formation stage. Further studies show an excessive activation of the PI3K/AKT/mTOR pathway in the hepatic tissues in the mutant, while treatment with the pathway inhibitor LY294002 and rapamycin partially rescues intrahepatic bile duct branching defects and alleviates the PBC-like symptoms. These findings implicate the potential role of the Ppp1r21-mediated PI3K/AKT/mTOR pathway in the pathophysiology of PBC.
