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Article

The pathogenesis of Niemann–Pick type C disease: a role for autophagy?

1 Sept 2008

Abstract excerpt

Niemann-Pick type C disease (NPC) is a sphingolipid-storage disorder that results from inherited deficiencies of intracellular lipid-trafficking proteins, and is characterised by an accumulation of cholesterol and glycosphingolipids in late endosomes and lysosomes. Patients with this disorder develop progressive neurological impairment that often begins in childhood, is ultimately fatal and is currently...

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PMID: 18782459
DOI: 10.1017/s146239940800080x

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