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

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

From Intrinsic Firing Properties to Selective Neuronal Vulnerability in Neurodegenerative Diseases

(2015) Francesco Roselli et al.   NEURON

Flocculus Purkinje cell signals in mouse Cacna1a calcium channel mutants of escalating severity: an investigation of the role of firing irregularity in ataxia

(2014) John S. Stahl et al.   JOURNAL OF NEUROPHYSIOLOGY

Overcoming Drug Development Bottlenecks With Repurposing: Old drugs learn new tricks

(2014) Stephen M Strittmatter   NATURE MEDICINE

Second Cistron in CACNA1A Gene Encodes a Transcription Factor Mediating Cerebellar Development and SCA6

(2013) Xiaofei Du et al.   CELL

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

Development of Purkinje cell degeneration in a knockin mouse model reveals lysosomal involvement in the pathogenesis of SCA6

(2012) T. Unno et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2011) R. Hourez 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

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 effect of 3,4-diaminopyridine on the patients with hereditary pure cerebellar ataxia

(2010) Taiji Tsunemi et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Sustained-release oral fampridine in multiple sclerosis: a randomised, double-blind, controlled trial

(2009) Andrew D Goodman et al.   LANCET

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

(2009) Alanna J Watt et al.   NATURE NEUROSCIENCE

Spinocerebellar ataxia type 6 knockin mice develop a progressive neuronal dysfunction with age-dependent accumulation of mutant CaV2.1 channels

(2008) K. Watase et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA