Article
Neuronal inclusions of α-synuclein contribute to the pathogenesis of Krabbe disease.
The Journal of pathology - 1 Apr 2014
Smith Benjamin R, Santos Marta B, Marshall Michael S, Cantuti-Castelvetri Ludovico, Lopez-Rosas Aurora, Li Guannan, van Breemen Richard, Claycomb Kumiko I, Gallea Jose I, Celej Maria S, Crocker Stephen J, Givogri Maria I, Bongarzone Ernesto R
Abstract excerpt
Demyelination is a major contributor to the general decay of neural functions in children with Krabbe disease. However, recent reports have indicated a significant involvement of neurons and axons in the neuropathology of the disease. In this study, we have investigated the nature of cellular inclusions in the Krabbe brain. Brain samples from the twitcher mouse model for Krabbe disease and from patients affected...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.
