Work

REEPing the benefits of an animal model of hereditary spastic paraplegia

Year: 2013

Type: article

Abstract: The hereditary spastic paraplegias (HSPs) are characterized by spasticity of the leg muscles due to axonal degeneration of corticospinal neurons. Beetz et al. report that the core motor phenotype and axonal pathology of HSPs are recapitulated in mice lacking the HSP-associated gene Reep1. REEP1 is shown to regulate ER structure in motor cortex neurons. The Reep1 knockout mouse should be a very useful model in which to study the mechanisms of progressive axon loss in HSPs and other disorders.

Source: Journal of Clinical Investigation

DOI: 10.1172/jci72324

Authors: Ariel Y. Deutch, Peter Hedera, Roger J. Colbran

Institutions: Vanderbilt University, Vanderbilt University Medical Center

Language: English

Topic: Hereditary Neurological Disorders

Subfield: Cellular and Molecular Neuroscience

Field: Neuroscience

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: bronze

Funders: Vanderbilt University, National Institutes of Health, National Institute of Mental Health, National Institute of Neurological Disorders and Stroke