期刊
FASEB JOURNAL
卷 21, 期 7, 页码 1492-1502出版社
WILEY
DOI: 10.1096/fj.06-7136com
关键词
survival of motoneuron; neurodegeneration; actin; PC12 cells
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 fulllength 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.
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