Mucopolysaccharidosis 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
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Organs or Tissue Humanized Mouse Models
Wild Mouse 765
Wild Mouse 765

Mucopolysaccharidosis (MPS) is a complex, progressive group of lysosomal storage disorders. It is characterized by multisystem involvement caused by deficiencies in enzymes that break down glycosaminoglycans (GAGs). Incomplete degradation of mucopolysaccharides leads to their lysosomal accumulation, resulting in diverse clinical manifestations such as facial abnormalities, nervous system issues, skeletal deformities, hepatosplenomegaly, heart disease, and corneal clouding. MPS has an estimated prevalence of around 1 in 100,000 people. Among its various types, MPS II is most prevalent in the Asian population, affecting about 1 in 160,000 male newborns, while MPS I is more commonly found in Europe and North America.

 

● Genetically Engineered MPS Mouse Models

Strain No.
 Strain Name Strain Type Description
T030220 Galns-KO Knock-out GALNS enzyme activity is not detected in the serum, liver, kidney, and spleen of Galns-KO homozygous mice, indicating an MPS type IVa-like phenotype
T056726 NCG-Idua-W392X Point mutation/knock-out IDUA activity is nearly undetectable in the serum, liver and heart of NCG-Idua-W392X homozygous mice, with significantly increased glycosaminoglycans in the urine, liver and heart compared to NCG wildtype mice
T011969 Ids-KO Knock-out IDS protein expression is rarely detected in homozygous Ids-KO mice. The glycosaminoglycans levels are significantly increased in the urine, liver and heart of homozygous mice at 17 weeks old compared to wildtype, and the bone density, especially the density and mineral content in the skull of homozygous mice, is dramatically increased by 13 weeks old. These phenotypes mimic the clinical pathology of MPS type II