White matter atrophy in spinocerebellar ataxia type 1 mouse models revealed by serial optical coherence scanner (Conference Presentation)

2018 ◽  
Author(s):  
Chao J. Liu ◽  
Harry T. Orr ◽  
Taner Akkin
Keyword(s):  
White Matter ◽  
Mouse Models ◽  
Spinocerebellar Ataxia ◽  
Spinocerebellar Ataxia Type ◽  
Optical Coherence ◽  
Spinocerebellar Ataxia Type 1

scholarly journals Mood alterations in mouse models of Spinocerebellar Ataxia type 1

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Melissa Asher ◽  
Juao-Guilherme Rosa ◽  
Marija Cvetanovic
Keyword(s):  
Cognitive Decline ◽  
Mouse Models ◽  
Spinocerebellar Ataxia ◽  
Cag Repeats ◽  
Spinocerebellar Ataxia Type ◽  
Motor Deficits ◽  
Brain Pathology ◽  
Spinocerebellar Ataxia Type 1 ◽  
Plus Maze

AbstractSpinocerebellar ataxia type 1 (SCA1) is a fatal neurodegenerative disease caused by abnormal expansion of glutamine-encoding CAG repeats in the Ataxin-1 (ATXN1) gene. SCA1 is characterized by progressive motor deficits, cognitive decline, and mood changes including anxiety and depression, with longer number of repeats correlating with worse disease outcomes. While mouse models have been very useful in understanding etiology of ataxia and cognitive decline, our understanding of mood symptoms in SCA1 has lagged. It remains unclear whether anxiety or depression stem from an underlying brain pathology or as a consequence of living with an untreatable and lethal disease. To increase our understanding of the etiology of SCA1 mood alterations, we used the elevated-plus maze, sucrose preference and forced swim tests to assess mood in four different mouse lines. We found that SCA1 knock-in mice exhibit increased anxiety that correlated with the length of CAG repeats, supporting the idea that underlying brain pathology contributes to SCA1-like anxiety. Additionally, our results support the concept that increased anxiety is caused by non-cerebellar pathology, as Purkinje cell specific SCA1 transgenic mice exhibit decreased anxiety-like behavior. Regarding the molecular mechanism, partial loss of ATXN1 may play a role in anxiety, based on our results for Atxn1 haploinsufficient and null mice.

scholarly journals Molecular pathway analysis towards understanding tissue vulnerability in spinocerebellar ataxia type 1

eLife ◽  
2018 ◽  
Vol 7 ◽  
Author(s):  
Terri M Driessen ◽  
Paul J Lee ◽  
Janghoo Lim
Keyword(s):  
Mouse Models ◽  
Spinocerebellar Ataxia ◽  
Inferior Olive ◽  
Neurodegenerative Disorder ◽  
Biological Pathways ◽  
Spinocerebellar Ataxia Type ◽  
Spinocerebellar Ataxia Type 1 ◽  
Molecular Alterations ◽  
First Time

The neurodegenerative disorder spinocerebellar ataxia type 1 (SCA1) affects the cerebellum and inferior olive, though previous research has focused primarily on the cerebellum. As a result, it is unknown what molecular alterations are present in the inferior olive, and whether these changes are found in other affected tissues. This study addresses these questions for the first time using two different SCA1 mouse models. We found that differentially regulated genes in the inferior olive segregated into several biological pathways. Comparison of the inferior olive and cerebellum demonstrates that vulnerable tissues in SCA1 are not uniform in their gene expression changes, and express largely discrete but some commonly enriched biological pathways. Importantly, we also found that brain-region-specific differences occur early in disease initiation and progression, and they are shared across the two mouse models of SCA1. This suggests different mechanisms of degeneration at work in the inferior olive and cerebellum.

Striatal dopamine nerve terminal markers but not nigral cellularity are reduced in spinocerebellar ataxia type 1

Neurology ◽  
1997 ◽  
Vol 48 (4) ◽  
pp. 1109-1111 ◽  
Author(s):  
S. J. Kish ◽  
M. Guttman ◽  
Y. Robitaille ◽  
M. El-Awar ◽  
L. -J. Chang ◽  
...  
Keyword(s):  
Spinocerebellar Ataxia ◽  
Nerve Terminal ◽  
Striatal Dopamine ◽  
Spinocerebellar Ataxia Type ◽  
Spinocerebellar Ataxia Type 1

Sequence variation and size ranges of CAG repeats in the Machado-Joseph disease, spinocerebellar ataxia type 1 and androgen receptor genes

1995 ◽  
Vol 4 (9) ◽  
pp. 1585-1590 ◽  
Author(s):  
David C. Rubinsztein ◽  
Jayne Leggo ◽  
Gerhard A. Coetzee ◽  
Ryan A. Irvine ◽  
Michael Buckley ◽  
...  
Keyword(s):  
Androgen Receptor ◽  
Spinocerebellar Ataxia ◽  
Sequence Variation ◽  
Cag Repeats ◽  
Spinocerebellar Ataxia Type ◽  
Spinocerebellar Ataxia Type 1 ◽  
Machado Joseph Disease

P124 Spinocerebellar ataxia type 1 – case report

2008 ◽  
Vol 119 ◽  
pp. S102-S103
Author(s):  
Svetlana Kostic ◽  
Dusko Vranjes ◽  
Velimir Dedic ◽  
Jagoda Potic
Keyword(s):  
Case Report ◽  
Spinocerebellar Ataxia ◽  
Spinocerebellar Ataxia Type ◽  
Spinocerebellar Ataxia Type 1

Maculopathy and Spinocerebellar Ataxia Type 1

2013 ◽  
Vol 33 (3) ◽  
pp. 225-231 ◽  
Author(s):  
Pierre Lebranchu ◽  
Guylène Le Meur ◽  
Armelle Magot ◽  
Albert David ◽  
Christophe Verny ◽  
...  
Keyword(s):  
Spinocerebellar Ataxia ◽  
Spinocerebellar Ataxia Type ◽  
Spinocerebellar Ataxia Type 1
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