Cargando…
Limited Phenotypic Effects of Selectively Augmenting the SMN Protein in the Neurons of a Mouse Model of Severe Spinal Muscular Atrophy
The selective vulnerability of motor neurons to paucity of Survival Motor Neuron (SMN) protein is a defining feature of human spinal muscular atrophy (SMA) and indicative of a unique requirement for adequate levels of the protein in these cells. However, the relative contribution of SMN-depleted mot...
Autores principales: | Lee, Andrew J-H., Awano, Tomoyuki, Park, Gyu-Hwan, Monani, Umrao R. |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2012
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3459898/ https://www.ncbi.nlm.nih.gov/pubmed/23029491 http://dx.doi.org/10.1371/journal.pone.0046353 |
Ejemplares similares
-
Muscle: an independent contributor to the neuromuscular spinal muscular atrophy disease phenotype
por: Jha, Narendra N., et al.
Publicado: (2023) -
Smn, the spinal muscular atrophy–determining gene product, modulates axon growth and localization of β-actin mRNA in growth cones of motoneurons
por: Rossoll, Wilfried, et al.
Publicado: (2003) -
Emerging concepts underlying selective neuromuscular dysfunction in infantile-onset spinal muscular atrophy
por: Gollapalli, Kishore, et al.
Publicado: (2021) -
A transgene carrying an A2G missense mutation in the SMN gene modulates phenotypic severity in mice with severe (type I) spinal muscular atrophy
por: Monani, Umrao R., et al.
Publicado: (2003) -
Effect of genetic background on the phenotype of the Smn(2B/-) mouse model of spinal muscular atrophy
por: Eshraghi, Mehdi, et al.
Publicado: (2016)