dc.creator | Ruiz Laza, Rocío | es |
dc.creator | Lin, J. | es |
dc.creator | Forgie, A. | es |
dc.creator | Foletti, D. | es |
dc.creator | Shelton, D. | es |
dc.creator | Rosenthal, A. | es |
dc.creator | Tabares, Lucía | es |
dc.date.accessioned | 2023-05-11T13:04:28Z | |
dc.date.available | 2023-05-11T13:04:28Z | |
dc.date.issued | 2005 | |
dc.identifier.citation | Ruiz Laza, R., Lin, J., Forgie, A., Foletti, D., Shelton, D., Rosenthal, A. y Tabares, L. (2005). Treatment with trkC agonist antibodies delays disease progression in neuromuscular degeneration (nmd) mice. HUMAN MOLECULAR GENETICS, 14 (13), 1825-1837. https://doi.org/10.1093/hmg/ddi189. | |
dc.identifier.issn | 0964-6906 | es |
dc.identifier.uri | https://hdl.handle.net/11441/145862 | |
dc.description.abstract | Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is a fatal autosomal recessive disorder
seen in infants. It is characterized by lower motor neuron degeneration, progressive muscle paralysis and
respiratory failure, for which no effective treatment exists. The phenotype of neuromuscular degeneration
(nmd ) mice closely resembles the human SMARD1. The identification of the mutated mouse gene in nmd
mice, Ighmbp2, led to the discovery of mutations of the homologous gene in humans with SMARD1. We
have studied the nmd mouse model with in vivo electrophysiological techniques and evaluated the efficacy
of Mab2256, a monoclonal antibody with agonist effect on the tyrosine kinase receptor C, trkC, on disease
progression in nmd mice. Treatment with Mab2256 resulted in a significant but transient improvement of
muscle strength in nmd mice, as well as normalization of the neuromuscular depression during highfrequency nerve stimulation. These results suggest the potential of using monoclonal agonist antibodies
for neurotrophin receptors in lower motor neuron diseases such as SMARD1. | es |
dc.format | application/pdf | es |
dc.format.extent | 13 p. | es |
dc.language.iso | eng | es |
dc.publisher | OXFORD UNIV PRESS | es |
dc.relation.ispartof | HUMAN MOLECULAR GENETICS, 14 (13), 1825-1837. | |
dc.rights | Attribution-NonCommercial-NoDerivatives 4.0 Internacional | * |
dc.rights.uri | http://creativecommons.org/licenses/by-nc-nd/4.0/ | * |
dc.title | Treatment with trkC agonist antibodies delays disease progression in neuromuscular degeneration (nmd) mice | es |
dc.type | info:eu-repo/semantics/article | es |
dcterms.identifier | https://ror.org/03yxnpp24 | |
dc.type.version | info:eu-repo/semantics/publishedVersion | es |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | es |
dc.contributor.affiliation | Universidad de Sevilla. Departamento de Bioquímica y Biología Molecular | es |
dc.contributor.affiliation | Universidad de Sevilla. Departamento de Fsiología Médica y Biofísica | es |
dc.relation.publisherversion | https://academic.oup.com/hmg/article/14/13/1825/621070 | es |
dc.identifier.doi | 10.1093/hmg/ddi189 | es |
dc.journaltitle | HUMAN MOLECULAR GENETICS | es |
dc.publication.volumen | 14 | es |
dc.publication.issue | 13 | es |
dc.publication.initialPage | 1825 | es |
dc.publication.endPage | 1837 | es |