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Article

Patterned Purkinje cell degeneration in mouse models of Niemann‐Pick type C disease

13 Jan 2003

Abstract excerpt

Niemann Pick disease type C1 (NPC1) is an inherited, autosomal recessive, lipid-storage disorder with major neurological involvement. Purkinje cell death is a prominent feature of the neuropathology of NPC. We have investigated Purkinje cell death in two murine models of NPC1, BALB/c npc(nih) and C57BLKS/J spm. In both cases, extensive Purkinje cell death was found in the cerebellum. The pattern of Purkinje cell...

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PMID: 12528192
DOI: 10.1002/cne.10522

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