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381 The role of leucine-rich PPR motif-containing protein (LRPPRC) in myelin lipid metabolism

OBJECTIVES/GOALS: Leigh Syndrome, French Canadian-Type (LSFC) is a neurometabolic disorder caused by mutation of mitochondria-related gene, LRPPRC. White matter lesions and demyelination in central nervous system are common in LSFC. LRPPRC is enriched in myelinating glial cells, yet its role is not...

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Detalles Bibliográficos
Autores principales: Palacios, Bridgitte, Ren, Jiangong, Hu, Jian
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cambridge University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10129471/
http://dx.doi.org/10.1017/cts.2023.417