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

2007 ◽  
Vol 21 (7) ◽  
pp. 1492-1502 ◽  
Author(s):  
Jeroen Bergeijk ◽  
Katharina Rydel‐Könecke ◽  
Claudia Grothe ◽  
Peter Claus
Keyword(s):  
Spinal Muscular Atrophy ◽  
Neurite Outgrowth ◽  
Gene Product ◽  
Muscular Atrophy ◽  
C Terminus

scholarly journals Osteoclast-stimulating Factor Interacts with the Spinal Muscular Atrophy Gene Product to Stimulate Osteoclast Formation

2001 ◽  
Vol 276 (44) ◽  
pp. 41035-41039 ◽  
Author(s):  
Noriyoshi Kurihara ◽  
Cheikh Menaa ◽  
Hidefumi Maeda ◽  
David J. Haile ◽  
Sakamuri V. Reddy
Keyword(s):  
Spinal Muscular Atrophy ◽  
Gene Product ◽  
Muscular Atrophy ◽  
Osteoclast Formation ◽  
Stimulating Factor

scholarly journals Smn, the spinal muscular atrophy–determining gene product, modulates axon growth and localization of β-actin mRNA in growth cones of motoneurons

2003 ◽  
Vol 163 (4) ◽  
pp. 801-812 ◽  
Author(s):  
Wilfried Rossoll ◽  
Sibylle Jablonka ◽  
Catia Andreassi ◽  
Ann-Kathrin Kröning ◽  
Kathrin Karle ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Gene Product ◽  
Muscular Atrophy ◽  
Axon Growth ◽  
Growth Cones ◽  
Binding Partner ◽  
Ribonucleoprotein Complexes ◽  
Small Nuclear Ribonucleoprotein ◽  
Actin Mrna ◽  
Nuclear Ribonucleoprotein

Spinal muscular atrophy (SMA), a common autosomal recessive form of motoneuron disease in infants and young adults, is caused by mutations in the survival motoneuron 1 (SMN1) gene. The corresponding gene product is part of a multiprotein complex involved in the assembly of spliceosomal small nuclear ribonucleoprotein complexes. It is still not understood why reduced levels of the ubiquitously expressed SMN protein specifically cause motoneuron degeneration. Here, we show that motoneurons isolated from an SMA mouse model exhibit normal survival, but reduced axon growth. Overexpression of Smn or its binding partner, heterogeneous nuclear ribonucleoprotein (hnRNP) R, promotes neurite growth in differentiating PC12 cells. Reduced axon growth in Smn-deficient motoneurons correlates with reduced β-actin protein and mRNA staining in distal axons and growth cones. We also show that hnRNP R associates with the 3′ UTR of β-actin mRNA. Together, these data suggest that a complex of Smn with its binding partner hnRNP R interacts with β-actin mRNA and translocates to axons and growth cones of motoneurons.

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