HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) All Versions of this Article: fj.06-7136comv1 21/7/1492    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by van Bergeijk, J. Articles by Claus, P. Search for Related Content PubMed PubMed Citation Articles by van Bergeijk, J. Articles by Claus, P.

The spinal muscular atrophy gene product regulates neurite outgrowth: importance of the C terminus

Jeroen van Bergeijk^*, Katharina Rydel-Könecke^*, Claudia Grothe^*, and Peter Claus^*,,1

^* Department of Neuroanatomy, Hannover Medical School, and

Center for Systems Neuroscience (ZSN) Hannover, Hannover, Germany

¹Correspondence: Department of Neuroanatomy, Hannover Medical School, OE 4140, Carl-Neuberg-Str.1, 30625 Hannover, Germany. E-mail: claus.peter{at}mh-hannover.de

Spinal muscular atrophy is a neurodegenerative disease accompanied by a loss of motoneurons. Either mutations or deletions in the survival of motoneuron (SMN) gene are responsible for this defect. SMN is an assembly protein for RNA-protein complexes in the nucleus and is also found in axons of neurons. However, it is unclear which dysfunctions of SMN are important for disease progression. In this study we analyzed the contributions of different SMN regions for localization and neuronal differentiation associated with outgrowth of neurites. Suppression of endogenous SMN protein levels significantly decreased the growth of neurites. Down-regulation of the interacting protein gemin2 had the opposite effect. Surprisingly, selective overexpression of the SMN C-terminal domain promoted neurite outgrowth similar to full-length protein and could rescue the SMN knock-down effects. The knock-down led to a significant change in the G-/F-actin ratio, indicating a role for SMN in actin dynamics. Therefore, our data suggest a functional role for SMN in microfilament metabolism in axons of motoneurons.—van Bergeijk, J., Rydel-Könecke, K., Grothe, C., Claus, P. The spinal muscular atrophy gene product regulates neurite outgrowth: importance of the C terminus.

Key Words: survival of motoneuron • neurodegeneration • actin • PC12 cells

This article has been cited by other articles:

				M. P. Walker, T.K. Rajendra, L. Saieva, J. L. Fuentes, L. Pellizzoni, and A. G. Matera SMN complex localizes to the sarcomeric Z-disc and is a proteolytic target of calpain Hum. Mol. Genet., November 1, 2008; 17(21): 3399 - 3410. [Abstract] [Full Text] [PDF]

				C. E. Beattie, T. L. Carrel, and M. L. McWhorter Fishing for a Mechanism: Using Zebrafish to Understand Spinal Muscular Atrophy J Child Neurol, August 1, 2007; 22(8): 995 - 1003. [Abstract] [PDF]