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Papers In Press, published online ahead of print September 23, 2003
Cellular Neurology Unit, NINDS, National Institutes of Health, Bethesda, MD 20892-4164
Corresponding Author: blackstc{at}ninds.nih.gov
Hereditary spastic paraplegias (HSPs) comprise a group of clinically heterogeneous syndromes characterized by lower extremity spasticity and weakness, with distal axonal degeneration in the long ascending and descending tracts of the spinal cord. The early-onset HSP SPG3A is caused by mutations in the atlastin/hGBP3 gene (renamed here atlastin1), which codes for a 64-kDa member of the dynamin/Mx/guanylate-binding protein (GBP) superfamily of large GTPases. The atlastin1 protein is localized predominantly in brain, where it is enriched in pyramidal neurons in the cerebral cortex and hippocampus. In cultured cortical neurons, atlastin1 co-localizes most prominently with markers of the Golgi apparatus, and immunogold electron microscopy reveals a predominant localization of atlastin1 to the cis-Golgi. Yeast two-hybrid analyses and co-immunoprecipitation studies demonstrate that atlastin1 can self-associate, and gel-exclusion chromatography and chemical cross-linking studies indicate that atlastin1 exists as an oligomer in vivo, most likely a tetramer. Membrane fractionation and protease protection assays reveal that atlastin1 is an integral membrane protein with two predicted transmembrane domains; both the N-terminal GTP-binding and C-terminal domains are exposed to the cytoplasm. Together these findings indicate that the SPG3A protein atlastin1 is a multimeric, integral membrane GTPase that may be involved in Golgi membrane dynamics or vesicle trafficking.
J. Biol. Chem, 10.1074/jbc.M306702200
Submitted on June 24, 2003
Revised on September 18, 2003
Accepted on September 23, 2003
Cellular Localization, Oligomerization, and Membrane Association of the Hereditary Spastic Paraplegia 3A (SPG3A) Protein Atlastin
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