Immortalized Mouse NPCNIH Schwann Cells (573C10)

Immortalized Mouse NPCNIH Schwann Cells (573C10) is a spontaneously immortalized cell line established from homozygous npcnih/npcnih mice. NPC1 is a gene responsible for the majority of Niemann–Pick disease type C (NPC) patients. NPC1 protein was not detectable in homozygous 573C10 cell. 573C10 Schwann cell line can be useful to investigate the pathomechanism of nervous system lesions in NPC, the therapeutic approaches against neurological deficits in patients with NPC and, more in general, the biology of cholesterol, sphingolipid and fatty acid transports in the nervous system.