Developmental defects and aberrant accumulation of endogenous psychosine in oligodendrocytes in a murine model of Krabbe disease

Developmental defects and aberrant accumulation of endogenous psychosine in oligodendrocytes in a murine model of Krabbe disease

Krabbe disease (KD), or globoid cell leukodystrophy, is an inherited lysosomal storage disease with leukodystrophy caused by a mutation in the galactosylceramidase (GALC) gene. The majority of patients show the early onset form of KD dominated by cerebral demyelination with apoptotic oligodendrocyte...

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Bibliographic Details
Main Authors: Naoko Inamura, Momoko Kito, Shinji Go, Soichiro Kishi, Masanori Hosokawa, Kiyofumi Asai, Nobuyuki Takakura, Hirohide Takebayashi, Junko Matsuda, Yasushi Enokido
Format: Article
Language:English
Published: Elsevier 2018-12-01
Series:Neurobiology of Disease
Subjects:
Krabbe disease
Oligodendrocyte
Demyelination
Leukodystrophy
Sphingolipidoses
Lysosomal storage disease
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996118305187

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http://www.sciencedirect.com/science/article/pii/S0969996118305187

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