title: Reduced levels of survival motor neuron protein leads to aberrant motoneuron growth in a Xenopus model of muscular atrophy.
abstract: 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.
authors.affiliation: CNRS UMR 8080, Laboratoire Développement, Morphogenèse et Evolution, 91405, Orsay, France.
authors.forename: Qods
authors.initials: Q
authors.lastname: Ymlahi-Ouazzani
authors.forename: Odile
authors.initials: O
authors.lastname: J Bronchain
authors.forename: Elodie
authors.initials: E
authors.lastname: Paillard
authors.forename: Chantal
authors.initials: C
authors.lastname: Ballagny
authors.forename: Albert
authors.initials: A
authors.lastname: Chesneau
authors.forename: Aurélie
authors.initials: A
authors.lastname: Jadaud
authors.forename: André
authors.initials: A
authors.lastname: Mazabraud
authors.forename: Nicolas
authors.initials: N
authors.lastname: Pollet
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D017209:Apoptosis; D001483:Base Sequence; D004195:Disease Models, Animal; D018507:Gene Expression Regulation, Developmental; D006801:Humans; D017403:In Situ Hybridization; D008969:Molecular Sequence Data; D009134:Muscular Atrophy, Spinal / Q000235:genetics*; D016376:Oligonucleotides, Antisense / Q000494:pharmacology; D011950:Receptors, Cholinergic / Q000378:metabolism; D020133:Reverse Transcriptase Polymerase Chain Reaction; D017386:Sequence Homology, Amino Acid; D055533:Survival of Motor Neuron 1 Protein / Q000096:biosynthesis*; D014981:Xenopus
chemical_list: D016376:Oligonucleotides, Antisense; D011950:Receptors, Cholinergic; D055533:Survival of Motor Neuron 1 Protein