Progressive Familial Intrahepatic Cholestasis Models
Obesity ModelsDiabetes ModelsAndrogenetic Alopecia (AGA) ModelsMASH ModelsChronic Liver Fibrosis ModelsAcute Liver Injury ModelsChronic Kindey Disease (CKD) ModelsCholestasis ModelsAlcohol-related Liver Disease (ALD) ModelsActue Kindey Disease (AKD) ModelsDiabetes Nephropathy (Diabetic Kidney Disease) ModelsOsteoporosis ModelsSarcopenia ModelsGout/Hyperuricemia ModelsFemale Reproduction Models
Hemophilia ModelsProgressive Familial Intrahepatic Cholestasis ModelsAutosomal Dominant Polycystic Kidney Disease (ADPKD)Glycogen Storage Disease type 1a ModelGrowth Failure ModelsFabry Disease ModelsHepatolenticular Degeneration ModelNiemann-Pick Disease (Sphingomyelinosis) ModelsHypophosphatasia ModelGM2 Gangliosidoses ModelPulmonary Alveolar Proteinosis ModelLimb Girdle Muscular Dystrophies ModelMaple Syrup Urine Disease ModelMucopolysaccharidosis ModelsHutchinson-Gilford Progeria Syndrome ModelPhenylketonuria/Hyperphenylalaninemia ModelsUrea Cycle Disorders ModelsTyrosinemia ModelThalassemia Models
Some autosomal recessive mutations in the bile acid membrane transporters affecting hepatocytes lead to progressive familial intrahepatic cholestasis (PFIC), a group of diseases characterized by cholestasis, pruritus, and often an eventual requirement for liver transplantation. The estimated incidence of PFIC ranges from 1 in 50,000 to 1 in 100,000 live births.
● Genetically Engineered PFIC Mouse Models
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Strain No.
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Strain Name | Strain Type | Description |
|---|---|---|---|
| T012640 | Nr1h4-KO | Knockout | Nr1h4-KO homozygous mice show impaired liver function and elevated serum TBA level. Lipid metabolism in this model is also impaired, as indicated by reduced serum TG and increased serum cholesterol levels |
| T007421 | Abcb4-KO | Knockout | Abcb4-KO homozygous mice show impaired liver function and development of liver fibrosis from 8 weeks old |