Patient fibroblasts-derived induced neurons demonstrate autonomous neuronal defects in adult-onset Krabbe disease

Krabbe disease (KD) is an autosomal recessive neurodegenerative disorder caused by defective β-galactosylceramidase (GALC), a lysosomal enzyme responsible for cleavage of several key substrates including psychosine. Accumulation of psychosine to the cytotoxic levels in KD patients is thought to caus...

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Detalhes bibliográficos
Publicado no:Oncotarget
Main Authors: Lim, Su Min, Choi, Byung-Ok, Oh, Seong-il, Choi, Won Jun, Oh, Ki-Wook, Nahm, Minyeop, Xue, Yuanchao, Choi, Jae Hyeok, Choi, Ji Young, Kim, Young-Eun, Chung, Ki Wha, Fu, Xiang-Dong, Ki, Chang-Seok, Kim, Seung Hyun
Formato: Artigo
Idioma:Inglês
Publicado em: Impact Journals LLC 2016
Assuntos:
Research Paper: Gerotarget (Focus on Aging)
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5342682/
https://ncbi.nlm.nih.gov/pubmed/27780934
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.18632/oncotarget.12812
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