Myocyte enhancer factor 2B （MEF2B） gene belongs to myocyte enhancer factor 2 （MEF2） gene family. They are all widely expressed in muscle and nerve tissues of human and animals. MEF2B plays an important role in the...Myocyte enhancer factor 2B （MEF2B） gene belongs to myocyte enhancer factor 2 （MEF2） gene family. They are all widely expressed in muscle and nerve tissues of human and animals. MEF2B plays an important role in the growth of muscle, development and differentiation of nerve system and liver fibrosis. This re- view mainly focused on the structural characteristics, tissue distribution, biological functions and research progress of MEF2B gene in human and animals.展开更多
Immunoglobulin G4-related sclerosing cholangitis （IgG4-SC） is an IgG4-related disease characterized by bile duct fibroinflammatory wall-thickening and stenosis, resulting in obstruction jaundice, weight loss. Dif- f...Immunoglobulin G4-related sclerosing cholangitis （IgG4-SC） is an IgG4-related disease characterized by bile duct fibroinflammatory wall-thickening and stenosis, resulting in obstruction jaundice, weight loss. Dif- ferent regions of the bile duct can be involved, with the distal region being the most common. IgG4-SC can also have other organ involvement, such as the pancreas, urinary tract, salivary glands and lacrimal glands. In clinical practice, the manifestation of IgG4-SC is very similar to cholangiocarcinoma （CC） and primary sclerosing cholangiUs （PSC）, as well as pancreatic malignancies, while the treatment and prognosis are totally different. Japanese researchers ever established the clinical diagnostic criteria in 2012： （1） charac- teristic biliary imaging findings; （2） elevated serum IgG4 concentrations; （3） the coexistence of IgG4-related diseases except those of the biliary tract; and （4） characteristic histopathological features. According to our observations, IgG4-SC can be distinguished from CC with 100% specificity only at a cutoff of six times the upper normal limit. Imaging findings have low specificity for diagnosis, with the exception of intraductal ultrasonography, which can reflect the lesion with relatively high specificity. IgG4 plasma cell infiltration can be found in bile duct biopsy tissue, although this procedure is difficult. According to recent studies, the treatment of IgG4-SC relies mainly on corticosteroids. Following steroid treatment, most IgG4-SC patients can recover and their symptoms are resolved although a few patients relapse after steroid withdrawal. Maintenance of steroid therapy or alternative drugs is necessary in such cases. There is, however, no stronq evidence for malignant transformation in I qG4-SC.展开更多
基金Supported by Financial Project from Ministry of Agriculture(2014-2130135)Specia Fund for Basal Scientific Research of Central Public-interest Scientific Institutes,Beijing Institute of Animal Husbandry and Veterinary,Chinese Academy of Agricultura Sciences(2014ywf-yb-7)~~
文摘Myocyte enhancer factor 2B （MEF2B） gene belongs to myocyte enhancer factor 2 （MEF2） gene family. They are all widely expressed in muscle and nerve tissues of human and animals. MEF2B plays an important role in the growth of muscle, development and differentiation of nerve system and liver fibrosis. This re- view mainly focused on the structural characteristics, tissue distribution, biological functions and research progress of MEF2B gene in human and animals.
文摘Immunoglobulin G4-related sclerosing cholangitis （IgG4-SC） is an IgG4-related disease characterized by bile duct fibroinflammatory wall-thickening and stenosis, resulting in obstruction jaundice, weight loss. Dif- ferent regions of the bile duct can be involved, with the distal region being the most common. IgG4-SC can also have other organ involvement, such as the pancreas, urinary tract, salivary glands and lacrimal glands. In clinical practice, the manifestation of IgG4-SC is very similar to cholangiocarcinoma （CC） and primary sclerosing cholangiUs （PSC）, as well as pancreatic malignancies, while the treatment and prognosis are totally different. Japanese researchers ever established the clinical diagnostic criteria in 2012： （1） charac- teristic biliary imaging findings; （2） elevated serum IgG4 concentrations; （3） the coexistence of IgG4-related diseases except those of the biliary tract; and （4） characteristic histopathological features. According to our observations, IgG4-SC can be distinguished from CC with 100% specificity only at a cutoff of six times the upper normal limit. Imaging findings have low specificity for diagnosis, with the exception of intraductal ultrasonography, which can reflect the lesion with relatively high specificity. IgG4 plasma cell infiltration can be found in bile duct biopsy tissue, although this procedure is difficult. According to recent studies, the treatment of IgG4-SC relies mainly on corticosteroids. Following steroid treatment, most IgG4-SC patients can recover and their symptoms are resolved although a few patients relapse after steroid withdrawal. Maintenance of steroid therapy or alternative drugs is necessary in such cases. There is, however, no stronq evidence for malignant transformation in I qG4-SC.
