Genetic Modeling of the Neurodegenerative Disease Spinocerebellar Ataxia Type 1 in Zebrafish

Genetic Modeling of the Neurodegenerative Disease Spinocerebellar Ataxia Type 1 in Zebrafish

Dominant spinocerebellar ataxias (SCAs) are progredient neurodegenerative diseases commonly affecting the survival of Purkinje cells (PCs) in the human cerebellum. Spinocerebellar ataxia type 1 (SCA1) is caused by the mutated <i>ataxin1</i> (<i>Atx1</i>) gene product, in whic...

Full description

Bibliographic Details
Main Authors:	Mohamed A. Elsaey, Kazuhiko Namikawa, Reinhard W. Köster
Format:	Article
Language:	English
Published:	MDPI AG 2021-07-01
Series:	International Journal of Molecular Sciences
Subjects:	spinocerebellar ataxia type 1 cerebellum polyglutamine disease neurodegeneration Purkinje cells bioimaging
Online Access:	https://www.mdpi.com/1422-0067/22/14/7351

Similar Items

Gene Deregulation and Underlying Mechanisms in Spinocerebellar Ataxias With Polyglutamine Expansion
by: Anna Niewiadomska-Cimicka, et al.
Published: (2020-06-01)

Autophagy is dysregulated in spinocerebellar ataxia type 2
by: Adriana Marcelo, et al.
Published: (2022-12-01)

Mutant Ataxin-2 Expression in Aged Animals Aggravates Neuropathological Features Associated with Spinocerebellar Ataxia Type 2
by: Inês T. Afonso, et al.
Published: (2022-10-01)

Experimental Treatment with Edaravone in a Mouse Model of Spinocerebellar Ataxia 1
by: Martina Sucha, et al.
Published: (2023-06-01)

Polyglutamine toxicity induces rod photoreceptor division, morphological transformation or death in Spinocerebellar ataxia 7 mouse retina
by: Marina G. Yefimova, et al.
Published: (2010-10-01)

Clinical and genetic profile in index patients with spinocerebellar ataxia type 3 in Indonesia: case report
by: Siti Aminah, et al.
Published: (2021-07-01)

Aberrant Cerebellar Circuitry in the Spinocerebellar Ataxias
by: Katherine J. Robinson, et al.
Published: (2020-07-01)

Cerebellar Development and Circuit Maturation: A Common Framework for Spinocerebellar Ataxias
by: Francesca Binda, et al.
Published: (2020-04-01)

Spinocerebellar ataxia type 40: A case report and literature review
by: Han Fengyue, et al.
Published: (2021-10-01)

Ophthalmic Manifestations and Genetics of the Polyglutamine Autosomal Dominant Spinocerebellar Ataxias: A Review
by: Jun Young Park, et al.
Published: (2020-08-01)

Collaborative Efforts for Spinocerebellar Ataxia Research in the United States: CRC-SCA and READISCA
by: Chih-Chun Lin, et al.
Published: (2020-08-01)

Pathogenetic Mechanisms Underlying Spinocerebellar Ataxia Type 3 Are Altered in Primary Oligodendrocyte Culture
by: Kristen H. Schuster, et al.
Published: (2022-08-01)

Spinocerebellar Ataxia Type 2: Clinicogenetic Aspects, Mechanistic Insights, and Management Approaches
by: Luis C. Velázquez-Pérez, et al.
Published: (2017-09-01)

A Novel Calpain Inhibitor Compound Has Protective Effects on a Zebrafish Model of Spinocerebellar Ataxia Type 3
by: Katherine J. Robinson, et al.
Published: (2021-09-01)

Transcriptome Profile of a New Mouse Model of Spinocerebellar Ataxia Type 14 Implies Changes in Cerebellar Development
by: Szilvia E. Mezey, et al.
Published: (2022-08-01)

Polyglutamine length-dependent toxicity from α1ACT in Drosophila models of spinocerebellar ataxia type 6
by: Wei-Ling Tsou, et al.
Published: (2016-12-01)

Viewpoint: spinocerebellar ataxias as diseases of Purkinje cell dysfunction rather than Purkinje cell loss
by: Josef P. Kapfhammer, et al.
Published: (2023-06-01)

In vivo assessment of neurodegeneration in Spinocerebellar Ataxia type 7
by: Jacob A. Parker, et al.
Published: (2021-01-01)

Neuronal vacuolation and spinocerebellar degeneration associated with altered neurotransmission
by: Aggeliki Giannakopoulou
Published: (2017-06-01)

Ataxin-3 protein modification as a treatment strategy for spinocerebellar ataxia type 3: Removal of the CAG containing exon
by: Melvin M. Evers, et al.
Published: (2013-10-01)

A Difficult Case to Diagnose: Machado-Joseph Disease/Spinocerebellar Ataxia Type III
by: Muhammad Sohail Ajmal Ghoauri, et al.
Published: (2023-12-01)

Cerebellar neuronal dysfunction accompanies early motor symptoms in spinocerebellar ataxia type 3
by: Kristin Mayoral-Palarz, et al.
Published: (2022-08-01)

Splice isoform-specific suppression of the CaV2.1 variant underlying spinocerebellar ataxia type 6
by: Wei-Ling Tsou, et al.
Published: (2011-09-01)

Population genetics of spinoсerebellar ataxias caused by polyglutamine expansions
by: A. N. Shuvaev, et al.
Published: (2019-07-01)

Neurocognitive and cerebellar function in ADHD, autism and spinocerebellar ataxia
by: Maurizio Cundari, et al.
Published: (2023-06-01)

Zebrafish Models of Rare Neurological Diseases like Spinocerebellar Ataxias (SCAs): Advantages and Limitations
by: Sreeja Sarasamma, et al.
Published: (2023-10-01)

Current Status of Gene Therapy Research in Polyglutamine Spinocerebellar Ataxias
by: Ricardo Afonso-Reis, et al.
Published: (2021-04-01)

Childhood-Onset Spinocerebellar Ataxia 3: Tongue Dystonia as an Early Manifestation
by: Nester Mitchell, et al.
Published: (2019-09-01)

Druggable genome screen identifies new regulators of the abundance and toxicity of ATXN3, the Spinocerebellar Ataxia type 3 disease protein
by: Naila S. Ashraf, et al.
Published: (2020-04-01)

Cognitive Changes in the Spinocerebellar Ataxias Due to Expanded Polyglutamine Tracts: A Survey of the Literature
by: Evelyn Lindsay, et al.
Published: (2017-07-01)

Research progress of spinocerebellar ataxia type 1
by: Lin-wei ZHANG, et al.
Published: (2014-05-01)

Occupational therapy in spinocerebellar ataxia type 3: an open-label trial
by: R.C.R. Silva, et al.
Published: (2010-06-01)

Pharmacological enhancement of retinoid-related orphan receptor α function mitigates spinocerebellar ataxia type 3 pathology
by: Masashi Watanave, et al.
Published: (2019-01-01)

Increased biological activity of protein Kinase C gamma is not required in Spinocerebellar ataxia 14
by: Etsuko Shimobayashi, et al.
Published: (2017-07-01)

Understanding the Pathophysiology of Spinocerebellar Ataxias through genetics, neurophysiology, structural and functional neuroimaging
by: Pramod Kumar Pal
Published: (2015-12-01)

Spinocerebellar Ataxia 27: A Review and Characterization of an Evolving Phenotype
by: Christopher L. Groth, et al.
Published: (2018-01-01)

Genetic Screening for Spinocerebellar Ataxia Genes in a Japanese Single-Hospital Cohort
by: Ryuji Sakakibara, et al.
Published: (2017-09-01)

Peripheral Oxidative Stress Biomarkers in Spinocerebellar Ataxia Type 3/Machado–Joseph Disease
by: Adriano M. de Assis, et al.
Published: (2017-09-01)

Computational neurobiology is a useful tool in translational neurology: the example of ataxia
by: Sherry-Ann eBrown, et al.
Published: (2015-01-01)

Recent Advancements in Targeted Delivery of Therapeutic Molecules in Neurodegenerative Disease - Spinocerebellar Ataxia - Opportunities and Challenges
by: Satya Prakash, et al.
Published: (2008-01-01)