40 Frozen Embryos of Tg (Npc1); BALB/c npcnih/nih. A Murine Model of Niemann-Pick C Disease
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NPC1 gene mutations result in Niemann-Pick disease type C (NPC) which is an autosomal recessive neurodegenerative disorder characterized by intracellular accumulation of cholesterol and gangliosides. The Niemann-Pick C deficient mouse model Npc1-/- recapitulates the disease phenotype of human patients. These mice however die around 8 weeks of age making it a difficult model to study the observed visceral cholesterol accumulation. NHGRI investigators generated a transgenic (Tg) (Npc1) mouse that when mated to the Npc1-/- model rescues the disease effects related to neurologic degeneration allowing for a normal lifespan. The new mouse strain defined as Tg (Npc1); BALB/c npcnih/nih rescues NPC1 disease but maintains the defects that correspond to the Niemann-Pick C visceral phenotype associated with cholesterol accumulation. 40 frozen embryos will be provided.
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