Evidence of Oxytosis/Ferroptosis in Niemann-Pick Disease Type C.

ABSTRACT: Niemann-Pick Disease Type C (NPC) is a hereditary neurodegenerative disease characterized by selective cell vulnerability, particularly affecting cerebellar anterior Purkinje neurons. These neurons exhibit a distinctive pattern of degeneration due to the loss of NPC1 and/or NPC2 protein function, progressively extending towards posterior cerebellar regions. Our study aimed to explore the early factors influencing this selective vulnerability of anterior Purkinje neurons in NPC. Oxytosis/ferroptosis, a novel form of regulated cell death, has been implicated in neurodegenerative diseases, with its inhibition showing promising therapeutic potential. Our laboratory has previously identified parallels between NPC cellular pathology and ferroptotic markers, including elevated levels of lipid per

SUBMITTER: Sanchez KL 

PROVIDER: S-EPMC11988824 | biostudies-literature | 2025 Mar

REPOSITORIES: biostudies-literature