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Front Neurosci. 2015 Jan 21;9:1. doi: 10.3389/fnins.2015.00001. eCollection 2015.

Computational neurobiology is a useful tool in translational neurology: the example of ataxia.

Author information

1
Department of Medicine, Mayo Clinic Rochester, MN, USA.
2
Departments of Neurology and Neuroscience, University of Connecticut Health Center Farmington, CT, USA.
3
Richard D. Berlin Center for Cell Analysis and Modeling, University of Connecticut Health Center Farmington, CT, USA.

Abstract

Hereditary ataxia, or motor incoordination, affects approximately 150,000 Americans and hundreds of thousands of individuals worldwide with onset from as early as mid-childhood. Affected individuals exhibit dysarthria, dysmetria, action tremor, and diadochokinesia. In this review, we consider an array of computational studies derived from experimental observations relevant to human neuropathology. A survey of related studies illustrates the impact of integrating clinical evidence with data from mouse models and computational simulations. Results from these studies may help explain findings in mice, and after extensive laboratory study, may ultimately be translated to ataxic individuals. This inquiry lays a foundation for using computation to understand neurobiochemical and electrophysiological pathophysiology of spinocerebellar ataxias and may contribute to development of therapeutics. The interdisciplinary analysis suggests that computational neurobiology can be an important tool for translational neurology.

KEYWORDS:

Purkinje; computational; homer; inositol 1,4,5-triphosphate receptor 1; model; neurology; spinocerebellar ataxia; translational

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