Neuroprotection and lifespan extension in Ppt1(−/−) mice by NtBuHA: therapeutic implications for INCL

Infantile neuronal ceroid lipofuscinosis (INCL) is a devastating childhood neurodegenerative lysosomal storage disease (LSD) that has no effective treatment. It is caused by inactivating mutations in the palmitoyl-protein thioesterase-1 (PPT1) gene. PPT1-deficiency impairs the cleavage of thioester...

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Detalles Bibliográficos
Autores principales: Sarkar, Chinmoy, Chandra, Goutam, Peng, Shyiong, Zhang, Zhongjian, Liu, Aiyi, Mukherjee, Anil B.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: 2013
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3812271/
https://www.ncbi.nlm.nih.gov/pubmed/24056696
http://dx.doi.org/10.1038/nn.3526

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3812271/
https://www.ncbi.nlm.nih.gov/pubmed/24056696
http://dx.doi.org/10.1038/nn.3526

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