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MBC in Press, published online ahead of print March 1, 2007
Mol. Biol. Cell 10.1091/mbc.E06-09-0833

A more recent version of this article appeared on May 1, 2007
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Submitted on September 18, 2006
Revised on January 30, 2007
Accepted on February 16, 2007

Troyer Syndrome Protein Spartin Is Mono-Ubiquitinated and Functions in EGF Receptor Trafficking

Joanna C. Bakowska,* Henri Jupille,* Parvin Fatheddin,* Rosa Puertollano,{dagger} and Craig Blackstone*

*Cellular Neurology Unit, National Institute of Neurological Disorders and Stroke, and {dagger}Laboratory of Cell Biology, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892

Monitoring Editor: Sandra Lemmon

Troyer syndrome is an autosomal recessive hereditary spastic paraplegia caused by mutation in the spartin (SPG20) gene, which encodes a widely-expressed protein of unknown function. This mutation results in premature protein truncation and thus might signify a loss-of-function disease mechanism. In this study, we have found that spartin is mono-ubiquitinated and functions in degradation of the epidermal growth factor receptor (EGFR). On EGF stimulation, spartin translocates from the cytoplasm to the plasma membrane and colocalizes with internalized EGF-Alexa. Knock-down of spartin by siRNA decreases the rate of EGFR degradation and also affects EGFR internalization, recycling, or both. Furthermore, overexpression of spartin results in a prominent decrease in EGFR degradation. Taken together, our data suggest that spartin is involved in the intracellular trafficking of EGFR and that impaired endocytosis may underlie the pathogenesis of Troyer syndrome.

Address correspondence to: Craig Blackstone (blackstc{at}ninds.nih.gov)




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Home page
 Arch NeurolHome page
J. C. Bakowska, H. Wang, B. Xin, C. J. Sumner, and C. Blackstone
Lack of Spartin Protein in Troyer Syndrome: A Loss-of-Function Disease Mechanism?
Arch Neurol, April 1, 2008; 65(4): 520 - 524.
[Abstract] [Full Text] [PDF]


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