Reduced levels of survival motor neuron protein leads to aberrant motoneuron growth in a Xenopus model of muscular atrophy
| Autor: | Odile Bronchain, Aurélie Jadaud, Elodie Paillard, Albert Chesneau, Chantal Ballagny, Nicolas Pollet, André Mazabraud, Qods Ymlahi-Ouazzani |
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| Přispěvatelé: | Développement et évolution (DE), Université Paris-Sud - Paris 11 (UP11)-Centre National de la Recherche Scientifique (CNRS), Neurobiologie & Développement (N&D), Centre National de la Recherche Scientifique (CNRS), Institut de Neurobiologie Alfred Fessard (INAF), Epigenomics Project, Genopole, Université d'Évry-Val-d'Essonne (UEVE) |
| Jazyk: | angličtina |
| Rok vydání: | 2010 |
| Předmět: |
Morpholino
MESH: Sequence Homology Amino Acid Xenopus Apoptosis SMN1 MESH: Amino Acid Sequence MESH: Base Sequence 0302 clinical medicine MESH: Oligonucleotides Antisense MESH: Muscular Atrophy Spinal MESH: Survival of Motor Neuron 1 Protein MESH: Reverse Transcriptase Polymerase Chain Reaction MESH: Gene Expression Regulation Developmental Receptors Cholinergic MESH: Animals MESH: Xenopus Amyotrophic lateral sclerosis In Situ Hybridization Genetics (clinical) 0303 health sciences biology Reverse Transcriptase Polymerase Chain Reaction Gene Expression Regulation Developmental Anatomy SMA medicine.anatomical_structure [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC] Molecular Sequence Data Muscular Atrophy Spinal 03 medical and health sciences Cellular and Molecular Neuroscience Atrophy MESH: In Situ Hybridization Genetics medicine Animals Humans Amino Acid Sequence 030304 developmental biology MESH: Humans MESH: Molecular Sequence Data MESH: Receptors Cholinergic Base Sequence Sequence Homology Amino Acid MESH: Apoptosis Spinal muscular atrophy Oligonucleotides Antisense Motor neuron medicine.disease biology.organism_classification Survival of Motor Neuron 1 Protein Disease Models Animal nervous system MESH: Disease Models Animal Neuroscience 030217 neurology & neurosurgery |
| Zdroj: | neurogenetics neurogenetics, Springer Verlag, 2010, 11 (1), pp.27-40. ⟨10.1007/s10048-009-0200-6⟩ neurogenetics, 2010, 11 (1), pp.27-40. ⟨10.1007/s10048-009-0200-6⟩ |
| ISSN: | 1364-6745 1364-6753 |
| DOI: | 10.1007/s10048-009-0200-6⟩ |
| Popis: | International audience; Spinal muscular atrophy (SMA) is a neurodegenerative disease characterized by motor neuron loss and skeletal muscle atrophy. The loss of function of the smn1 gene, the main supplier of survival motor neuron protein (SMN) protein in human, leads to reduced levels of SMN and eventually to SMA. Here, we ask if the amphibian Xenopus tropicalis can be a good model system to study SMA. Inhibition of the production of SMN using antisense morpholinos leads to caudal muscular atrophy in tadpoles. Of note, early developmental patterning of muscles and motor neurons is unaffected in this system as well as acetylcholine receptors clustering. Muscular atrophy seems to rather result from aberrant pathfinding and growth arrest and/or shortening of motor axons. This event occurs in the absence of neuronal cell bodies apoptosis, a process comparable to that of amyotrophic lateral sclerosis. Xenopus tropicalis is revealed as a complementary animal model for the study of SMA. |
| Databáze: | OpenAIRE |
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