Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding.
Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding.
复制标题
DOI：
10.1083/jcb.200303168
复制
发表时间：
2003-09-01
期刊：
影响因子：
--
通讯作者：
Beattie CE
中科院分区：
文献类型：
--
作者：
McWhorter ML;Monani UR;Burghes AH;Beattie CE
Spinal muscular atrophy (SMA) is an autosomal recessive disorder characterized by a loss of α motoneurons in the spinal cord. SMA is caused by low levels of the ubiquitously expressed survival motor neuron (Smn) protein. As it is unclear how low levels of Smn specifically affect motoneurons, we have modeled SMA in zebrafish, a vertebrate model organism with well-characterized motoneuron development. Using antisense morpholinos to reduce Smn levels throughout the entire embryo, we found motor axon–specific pathfinding defects. Reduction of Smn in individual motoneurons revealed that smn is acting cell autonomously. These results show for the first time, in vivo, that Smn functions in motor axon development and suggest that these early developmental defects may lead to subsequent motoneuron loss.
登录
查看更多内容
影响因子：
3.5
作者：
Jablonka, S;Bandilla, M;Fischer, U
通讯作者：
Fischer, U
影响因子：
3.4
作者：
Bromberg, MB;Swoboda, KJ
通讯作者：
Swoboda, KJ
影响因子：
3.5
作者：
Coovert, DD;Le, TT;Burghes, AHM
通讯作者：
Burghes, AHM
影响因子：
3.8
作者：
Beattie, CE
通讯作者：
Beattie, CE
影响因子：
30.8
作者：
Cartegni, L;Krainer, AR
通讯作者：
Krainer, AR