[P103]: Establishment of spinocerebellar ataxia type 8 transgenic mouse model

2006 ◽  
Vol 24 (8) ◽  
pp. 539-540
Author(s):  
H.Y. Lin ◽  
C.S. Hsu ◽  
H.M. Hsieh-Li ◽  
C.M. Chen ◽  
K.T. Lu ◽  
...  
Keyword(s):  
Mouse Model ◽  
Transgenic Mouse ◽  
Spinocerebellar Ataxia ◽  
Transgenic Mouse Model ◽  
Spinocerebellar Ataxia Type

scholarly journals Altered striatal endocannabinoid signaling in a transgenic mouse model of spinocerebellar ataxia type-3

PLoS ONE ◽  
2017 ◽  
Vol 12 (4) ◽  
pp. e0176521 ◽  
Author(s):  
Carmen Rodríguez-Cueto ◽  
Mariluz Hernández-Gálvez ◽  
Cecilia J. Hillard ◽  
Patricia Maciel ◽  
Sara Valdeolivas ◽  
...  
Keyword(s):  
Mouse Model ◽  
Transgenic Mouse ◽  
Spinocerebellar Ataxia ◽  
Transgenic Mouse Model ◽  
Spinocerebellar Ataxia Type ◽  
Spinocerebellar Ataxia Type 3 ◽  
Type 3

scholarly journals Dysregulation of the endocannabinoid signaling system in the cerebellum and brainstem in a transgenic mouse model of spinocerebellar ataxia type-3

Neuroscience ◽  
2016 ◽  
Vol 339 ◽  
pp. 191-209 ◽  
Author(s):  
Carmen Rodríguez-Cueto ◽  
Mariluz Hernández-Gálvez ◽  
Cecilia J. Hillard ◽  
Patricia Maciel ◽  
Luis García-García ◽  
...  
Keyword(s):  
Mouse Model ◽  
Transgenic Mouse ◽  
Spinocerebellar Ataxia ◽  
Transgenic Mouse Model ◽  
Spinocerebellar Ataxia Type ◽  
Signaling System ◽  
Spinocerebellar Ataxia Type 3 ◽  
Type 3

Caffeine alleviates progressive motor deficits in a transgenic mouse model of spinocerebellar ataxia

2017 ◽  
Vol 81 (3) ◽  
pp. 407-418 ◽  
Author(s):  
Nélio Gonçalves ◽  
Ana T. Simões ◽  
Rui D. Prediger ◽  
Hirokazu Hirai ◽  
Rodrigo A. Cunha ◽  
...  
Keyword(s):  
Mouse Model ◽  
Transgenic Mouse ◽  
Spinocerebellar Ataxia ◽  
Transgenic Mouse Model ◽  
Motor Deficits ◽  
Progressive Motor Deficits

scholarly journals Reactive astrocytosis and glial glutamate transporter clustering are early changes in a spinocerebellar ataxia type 1 transgenic mouse model

2007 ◽  
Vol 3 (4) ◽  
pp. 335-351 ◽  
Author(s):  
Roberto Giovannoni ◽  
Nicola Maggio ◽  
Maria Rosaria Bianco ◽  
Carlo Cavaliere ◽  
Giovanni Cirillo ◽  
...  
Keyword(s):  
Mouse Model ◽  
Synaptic Transmission ◽  
Transgenic Mouse ◽  
Glutamate Transporter ◽  
Transgenic Mouse Model ◽  
Spinocerebellar Ataxia Type ◽  
Spinocerebellar Ataxia Type 1 ◽  
Glial Glutamate Transporter ◽  
Reactive Astrocytosis

AbstractSpinocerebellar ataxia type 1 (SCA1) is a neurodegenerative disorder caused by an expanded CAG trinucleotide repeats within the coding sequence of the ataxin-1 protein. In the present study, we used a conditional transgenic mouse model of SCA1 to investigate very early molecular and morphological changes related to the behavioral phenotype. In mice with neural deficits detected by rotarod performance, and simultaneous spatial impairments in exploratory activity and uncoordinated gait, we observed both significant altered expression and patchy distribution of excitatory amino acids transporter 1. The molecular changes observed in astroglial compartments correlate with changes in synapse morphology; synapses have a dramatic reduction of the synaptic area external to the postsynaptic density. By contrast, Purkinje cells demonstrate preserved structure. In addition, severe reactive astrocytosis matches changes in the glial glutamate transporter and synapse morphology. We propose these morpho-molecular changes are the cause of altered synaptic transmission, which, in turn, determines the onset of the neurological symptoms by altering the synaptic transmission in the cerebellar cortex of transgenic animals. This model might be suitable for testing drugs that target activated glial cells in order to reduce CNS inflammation.

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