Altered synaptic and firing properties of cerebellar Purkinje cells in a mouse model of ARSACS

Antisense oligonucleotide therapy for spinocerebellar ataxia type 2

(2017) Daniel R. Scoles et al.   NATURE

The dialectic of Hebb and homeostasis

(2017) Gina G. Turrigiano   PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES

Prolonged Type 1 Metabotropic Glutamate Receptor Dependent Synaptic Signaling Contributes to Spino-Cerebellar Ataxia Type 1

(2016) Emmet M. Power et al.   JOURNAL OF NEUROSCIENCE

New old drug(s) for spinocerebellar ataxias

(2016) Visou Ady et al.   JOURNAL OF PHYSIOLOGY-LONDON

Cellular and circuit mechanisms underlying spinocerebellar ataxias

(2016) Pratap Meera et al.   JOURNAL OF PHYSIOLOGY-LONDON

Progressive impairment of cerebellar mGluR signalling and its therapeutic potential for cerebellar ataxia in spinocerebellar ataxia type 1 model mice

(2016) Anton N. Shuvaev et al.   JOURNAL OF PHYSIOLOGY-LONDON

Transient cerebellar alterations during development prior to obvious motor phenotype in a mouse model of spinocerebellar ataxia type 6

(2016) Sriram Jayabal et al.   JOURNAL OF PHYSIOLOGY-LONDON

4-aminopyridine reverses ataxia and cerebellar firing deficiency in a mouse model of spinocerebellar ataxia type 6

(2016) Sriram Jayabal et al.   Scientific Reports

Structural and functional MRI abnormalities of cerebellar cortex and nuclei in SCA3, SCA6 and Friedreich’s ataxia

(2015) Maria R. Stefanescu et al.   BRAIN

Docosahexaenoic acid (DHA): a modulator of microglia activity and dendritic spine morphology

(2015) Philip Chang et al.   Journal of Neuroinflammation

In vivo analysis of Purkinje cell firing properties during postnatal mouse development

(2015) Marife Arancillo et al.   JOURNAL OF NEUROPHYSIOLOGY

Spinocerebellar Ataxia Type 6 Protein Aggregates Cause Deficits in Motor Learning and Cerebellar Plasticity

(2015) M. D. Mark et al.   JOURNAL OF NEUROSCIENCE

Neuronal Atrophy Early in Degenerative Ataxia Is a Compensatory Mechanism to Regulate Membrane Excitability

(2015) J. M. Dell'Orco et al.   JOURNAL OF NEUROSCIENCE

Synapse-specific expression of calcium-permeable AMPA receptors in neocortical layer 5

(2015) Txomin Lalanne et al.   JOURNAL OF PHYSIOLOGY-LONDON

High-Throughput Screening for Ligands of the HEPN Domain of Sacsin

(2015) Xinlu Li et al.   PLoS One

Diversity of ARSACS Mutations in French-Canadians

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Prolonged ampakine exposure prunes dendritic spines and increases presynaptic release probability for enhanced long-term potentiation in the hippocampus

(2014) Philip K.-Y. Chang et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Sacs knockout mice present pathophysiological defects underlying autosomal recessive spastic ataxia of Charlevoix-Saguenay

(2014) Roxanne Larivière et al.   HUMAN MOLECULAR GENETICS

Differential GABAergic and Glycinergic Inputs of Inhibitory Interneurons and Purkinje Cells to Principal Cells of the Cerebellar Nuclei

(2014) Z. Husson et al.   JOURNAL OF NEUROSCIENCE

Autosomal recessive spastic ataxia of Charlevoix Saguenay (ARSACS): expanding the genetic, clinical and imaging spectrum

(2013) Matthis Synofzik et al.   Orphanet Journal of Rare Diseases

Selective Positive Modulator of Calcium-Activated Potassium Channels Exerts Beneficial Effects in a Mouse Model of Spinocerebellar Ataxia Type 2

(2012) Adebimpe W. Kasumu et al.   CHEMISTRY & BIOLOGY

Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2

(2012) S. T. Hansen et al.   HUMAN MOLECULAR GENETICS

Mitochondrial dysfunction and Purkinje cell loss in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS)

(2012) M. Girard et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Patterned Purkinje cell loss in the ataxic sticky mouse

(2011) Justyna R. Sarna et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Aminopyridines Correct Early Dysfunction and Delay Neurodegeneration in a Mouse Model of Spinocerebellar Ataxia Type 1

(2011) R. Hourez et al.   JOURNAL OF NEUROSCIENCE

Delayed Postnatal Loss of P/Q-Type Calcium Channels Recapitulates the Absence Epilepsy, Dyskinesia, and Ataxia Phenotypes of Genomic Cacna1A Mutations

(2011) M. D. Mark et al.   JOURNAL OF NEUROSCIENCE

Early Changes in Cerebellar Physiology Accompany Motor Dysfunction in the Polyglutamine Disease Spinocerebellar Ataxia Type 3

(2011) V. G. Shakkottai et al.   JOURNAL OF NEUROSCIENCE

Lobule-specific membrane excitability of cerebellar Purkinje cells

(2011) Chang-Hee Kim et al.   JOURNAL OF PHYSIOLOGY-LONDON

Purkinje neuron synchrony elicits time-locked spiking in the cerebellar nuclei

(2011) Abigail L. Person et al.   NATURE

Cerebellar Zones: A Personal History

(2010) Jan Voogd   CEREBELLUM

The Therapeutic Mode of Action of 4-Aminopyridine in Cerebellar Ataxia

(2010) K. Alvina et al.   JOURNAL OF NEUROSCIENCE

KCa Channels as Therapeutic Targets in Episodic Ataxia Type-2

(2010) K. Alvina et al.   JOURNAL OF NEUROSCIENCE

The ataxia protein sacsin is a functional co-chaperone that protects against polyglutamine-expanded ataxin-1

(2009) David A. Parfitt et al.   HUMAN MOLECULAR GENETICS

Traveling waves in developing cerebellar cortex mediated by asymmetrical Purkinje cell connectivity

(2009) Alanna J Watt et al.   NATURE NEUROSCIENCE

FGF14 regulates the intrinsic excitability of cerebellar Purkinje neurons

(2008) Vikram G. Shakkottai et al.   NEUROBIOLOGY OF DISEASE