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Differentiation defects in primary motoneurons from a SMARD1 mouse model that are insensitive to treatment with low dose PEGylated IGF1
Muscle atrophy and diaphragmatic palsy are the clinical characteristics of spinal muscular atrophy with respiratory distress type 1 (SMARD1), and are well represented in the neuromuscular degeneration (Nmd(2J)) mouse, modeling the juvenile form of SMARD1. Both in humans and mice mutations in the IGH...
Autores principales: | Krieger, Frank, Metzger, Friedrich, Jablonka, Sibylle |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Landes Bioscience
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4116388/ https://www.ncbi.nlm.nih.gov/pubmed/25083343 http://dx.doi.org/10.4161/rdis.29415 |
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