Cargando…
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...
| Autores principales: | , , , , , |
|---|---|
| Formato: | Online Artículo Texto |
| Lenguaje: | English |
| Publicado: |
2013
|
| Materias: | |
| 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 |