NDLI: REEPing the benefits of an animal model of hereditary spastic paraplegia.

Content Provider	Semantic Scholar
Author	Deutch, Ariel Y. Hedera, Peter Colbran, Roger J.
Copyright Year	2013
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.
Starting Page	792
Ending Page	806
Page Count	15
File Format	PDF HTM / HTML
Alternate Webpage(s)	http://dm5migu4zj3pb.cloudfront.net/manuscripts/72000/72324/JCI72324.v2.pdf
PubMed reference number	24051371v1
Alternate Webpage(s)	https://doi.org/10.1172/JCI72324
DOI	10.1172/jci72324
Journal	The Journal of clinical investigation
Volume Number	123
Issue Number	10
Language	English
Access Restriction	Open
Subject Keyword	Heat shock proteins Mice, Knockout Muscle Spasticity REEP1 gene Sensorineural Hearing Loss (disorder) Spastic Paraplegia Spastic Paraplegia, Hereditary Tropical Spastic Paraparesis benefit primary motor cortex
Content Type	Text
Resource Type	Article

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REEPing the benefits of an animal model of hereditary spastic paraplegia.