The blood-brain barrier is disrupted in Machado-Joseph disease/spinocerebellar ataxia type 3: evidence from transgenic mice and human post-mortem samples

The blood-brain barrier is disrupted in Machado-Joseph disease/spinocerebellar ataxia type 3: evidence from transgenic mice and human post-mortem samples

Abstract Blood-brain barrier (BBB) disruption is a common feature in neurodegenerative diseases. However, BBB integrity has not been assessed in spinocerebellar ataxias (SCAs) such as Machado-Joseph disease/SCA type 3 (MJD/SCA3), a genetic disorder, triggered by polyglutamine-expanded ataxin-3. To i...

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Bibliographic Details
Main Authors: Diana Duarte Lobo, Rui Jorge Nobre, Catarina Oliveira Miranda, Dina Pereira, João Castelhano, José Sereno, Arnulf Koeppen, Miguel Castelo-Branco, Luís Pereira de Almeida
Format: Article
Language:English
Published: BMC 2020-08-01
Series:Acta Neuropathologica Communications
Subjects:
Machado-Joseph disease (MJD)
Spinocerebellar Ataxia type 3 (SCA3)
Blood-brain barrier (BBB)
Tight junctions (TJ)
Dynamic contrast enhanced-magnetic resonance imaging (DCE-MRI)
Online Access:http://link.springer.com/article/10.1186/s40478-020-00955-0

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http://link.springer.com/article/10.1186/s40478-020-00955-0

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