huHSC-NCG-X

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

Proto-oncogene c-KIT, also known as CD117 (cluster of differentiation 117 or mast/ stem cell growth factor receptor (SCFR)), is a receptor tyrosine kinase protein that is encoded by the KIT gene. Generally, hematopoietic stem/progenitor cells express c-kit, while acute lymphoblastic leukemia cells rarely express it. The c-KIT protein plays a crucial function in the signaling pathway for bone marrow-to-plasma migration of hematopoietic progenitor cells. 

C-KIT mutations have been linked to multiple types of cancer. The W41 is a point mutation at position 831 Val to Met in the c-kit protein, which may  suppress the function of hematopoietic stem cells, resulting in spontaneous anemia in mice.

GemPharmatech developed the NCG-X strain, which does not require irradiation during HSC reconstitution, and huHSC-NCG-X can reconstitute red lineage cells in mouse bone marrow that could be utilized to research thalassemia.


Applications

  • Human immune system development

  • Research on thalassemia

  • Evaluation of anti-tumor drugs targeting red blood cells

  • Immune system reconstitution models that do not require irradiation or chemoablation

 

Supporting data

1. Human immune reconstitution levels of huHSC-NCG-X mice

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Compared with irradiated NCG mice, huHSC-NCG-X mice were able to rebuild the human immune system efficiently without irradiation.


2. Reconstitution levels of red blood cells in huHSC-NCG-X mice in the bone marrow

图片2.png

The human HSC reconstruction assays in NCG-X mice demonstrated a considerable increase in the proportion of reconstructed erythrocytes in the bone marrow of NCG-X mice compared to irradiated NCG mice. This finding suggests that huHSC-NCG-X can be used as a good model to research diseases like thalassemia.


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