Changes in structure and function of diaphragm neuromuscular junctions from BACHD mouse model for Huntington's disease

2016 ◽  
Vol 93 ◽  
pp. 64-72 ◽  
Author(s):  
Bárbara Campos de Aragão ◽  
Hermann Alecsandro Rodrigues ◽  
Priscila Aparecida Costa Valadão ◽  
Wallace Camargo ◽  
Lígia Araujo Naves ◽  
...  
Keyword(s):  
Mouse Model ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neuromuscular Junctions ◽  
Structure And Function ◽  
And Function

scholarly journals Abnormalities in the Motor Unit of a Fast-Twitch Lower Limb Skeletal Muscle in Huntington’s Disease

ASN NEURO ◽  
2019 ◽  
Vol 11 ◽  
pp. 175909141988621 ◽  
Author(s):  
Priscila Aparecida Costa Valadão ◽  
Bárbara Campos de Aragão ◽  
Jéssica Neves Andrade ◽  
Matheus Proença S. Magalhães-Gomes ◽  
Giselle Foureaux ◽  
...  
Keyword(s):  
Mouse Model ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Tibialis Anterior ◽  
Neuromuscular Junctions ◽  
Motor Behavior ◽  
Psychiatric Symptoms ◽  
Artificial Chromosome ◽  
Ultrastructural Level ◽  
Fast Twitch

Huntington’s disease (HD) is a disorder characterized by chronic involuntary movements, dementia, and psychiatric symptoms. It is caused by a mutation in the gene that encodes for huntingtin protein (HTT), leading to the formation of mutant proteins expressed in various tissues. Although brain pathology has become the hallmark for HD, recent studies suggest that damage of peripheral structures also contributes to HD progression. We previously identified severe alterations in the motor units that innervate cervical muscles in 12-month-old BACHD (Bacterial Artificial Chromosome Huntington’s Disease) mice, a well-established mouse model for HD. Here, we studied lumbar motoneurons and their projections onto hind limb fast-twitch skeletal muscles (tibialis anterior), which control balance and gait in HD patients. We found that lumbar motoneurons were altered in the HD mouse model; the number and size of lumbar motoneurons were reduced in BACHD. Structural alterations were also present in the sciatic nerve and neuromuscular junctions. Acetylcholine receptors were organized in several small patches (acetylcholine receptor fragmentation), many of which were partially innervated. In BACHD mice, we observed atrophy of tibialis anterior muscles, decreased expression of glycolytic fast Type IIB fibers, and at the ultrastructural level, alterations of sarcomeres and mitochondria. Corroborating all these findings, BACHD animals performed worse on motor behavior tests. Our results provide additional evidences that nerve–muscle communication is impaired in HD and that motoneurons from distinct spinal cord locations are similarly affected in the disease.

The impact of bilingualism on brain structure and function in Huntington's disease

2019 ◽  
Vol 60 ◽  
pp. 92-97 ◽  
Author(s):  
Saul Martínez-Horta ◽  
Andrea Moreu ◽  
Jesús Perez-Perez ◽  
Frederic Sampedro ◽  
Andrea Horta-Barba ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Brain Structure ◽  
Structure And Function ◽  
Brain Structure And Function ◽  
And Function ◽  
The Impact

Motor network structure and function are associated with motor performance in Huntington’s disease

2016 ◽  
Vol 263 (3) ◽  
pp. 539-549 ◽  
Author(s):  
Hans-Peter Müller ◽  
Martin Gorges ◽  
Georg Grön ◽  
Jan Kassubek ◽  
G. Bernhard Landwehrmeyer ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Network Structure ◽  
Motor Performance ◽  
Structure And Function ◽  
Motor Network ◽  
And Function

scholarly journals Intact sensory-motor network structure and function in far from onset premanifest Huntington’s disease

2017 ◽  
Vol 7 (1) ◽  
Author(s):  
Martin Gorges ◽  
Hans-Peter Müller ◽  
Isabella Maria Sophie Mayer ◽  
Gesa Sophie Grupe ◽  
Thomas Kammer ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Network Structure ◽  
Structure And Function ◽  
Motor Network ◽  
Sensory Motor ◽  
And Function

E04 Structure and function of the posterior cerebral cortex in huntington’s disease

2018 ◽  
Author(s):  
Emma M Coppen ◽  
Anne Hafkemeijer ◽  
Jeroen van der Grond ◽  
Jurriaan JH Barkey Wolf ◽  
Raymund AC Roos
Keyword(s):  
Cerebral Cortex ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Structure And Function ◽  
And Function
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