Progressive Familial Intrahepatic Cholestasis Models

Autoimmune Disease Models
Autoimmune Nephropathy ModelsAsthma ModelsSystemic Lupus Erythematosus (SLE) ModelsSjögren's Syndrome ModelsAutoimmune Dermatoses Models Neuroimmune Disease ModelsInflammatory Bowel Disease (IBD) ModelsRheumatoid Arthritis ModelsImmune Reconstitution Autoimmune Disease Models
Metabolic Disease Mouse 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
Wild Mouse 750
Wild Mouse 750
Rare Disease Mouse 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
Neurological Disease Mouse Models
Alzheimer's Disease (AD) ModelsHumanized Alzheimer's Disease (AD) ModelsParkinson's Disease (PD) ModelsHuntington's Disease (HD) ModelsAmyotrophic Lateral Sclerosis (ALS) ModelsTransthyretin Amyloidosis (ATTR) ModelsPain Mouse ModelsDuchenne Muscular Dystrophy (DMD) Models
Oncology Mouse Models
Human Xenograft ModelsDrug ResistanceT-Cell EngagersHumanized Immune Checkpoint ModelsHumanized Immune System ModelsSpontaneous Tumor Models
Cardiovascular Disease Mouse Models
Heart Failure ModelsHypertension ModelsAtherosclerosis ModelsAdvanced Cardiovascular and Metabolic Complication ModelsHyperlipidemia ModelsHypertropic Cardiomyopathy ModelsStroke ModelsThrombosis Models
Humanized Immune System Models
huPBMC-NCGhuHSC-NCG-XhuHSC-NCG-hIL15huHSC-NCG
Organs or Tissue Humanized Mouse Models
Wild Mouse 765
Wild Mouse 765

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

Strain No.
 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