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| DOI | 10.1002/TERM.2626 | ||||
| Año | 2018 | ||||
| Tipo | artículo de investigación |
Citas Totales
Autores Afiliación Chile
Instituciones Chile
% Participación
Internacional
Autores
Afiliación Extranjera
Instituciones
Extranjeras
Ataxias are locomotor disorders that can have an origin both neural and muscular, although both impairments are related. Unfortunately, ataxia has no cure, and the current therapies are aimed at motor re-education or muscular reinforcement. Nevertheless, cell therapy is becoming a promising approach to deal with incurable neural diseases, including neuromuscular ataxias. Here, we have used a model of ataxia, the Purkinje Cell Degeneration (PCD) mutant mouse, to study the effect of healthy (wild-type) bone marrow transplantation on the restoration of defective mobility. Bone marrow transplants (from both mutant and healthy donors) were performed in wild-type and PCD mice. Then, a wide battery of behavioural tests was employed to determine possible motor amelioration in mutants. Finally, cerebellum, spinal cord, and muscle were analysed to study the integration of the transplant-derived cells and the origin of the behavioural changes. Our results demonstrated that the transplant of wild-type bone marrow restores the mobility of PCD mice, increasing their capabilities of movement (52-100% of recovery), exploration (20-71% of recovery), speed (35% of recovery), and motor coordination (25% of recovery). Surprisingly, our results showed that bone marrow transplant notably improves the skeletal muscle structure, which is severely damaged in the mutants, rather than ameliorating the central nervous system. Although a multimodal effect of the transplant is not discarded, muscular improvements appear to be the basis of this motor recovery. Furthermore, the results from our study indicate that bone marrow stem cell therapy can be a safe and effective alternative for dealing with movement disorders such as ataxias.
| Ord. | Autor | Género | Institución - País |
|---|---|---|---|
| 1 | Diaz, D. | Hombre |
Hospital de Salamanca - España
IBSAL - España Instituto de Neurociencias de Castilla y León - España Instituto de Investigación Biomédica de Salamanca (IBSAL) - España Univ Salamanca - España |
| 2 | Piquer-Gil, Marina | Mujer |
CSIC - España
Consejo Superior de Investigaciones Científicas - España CSIC-JA-UPO-USE - Centro Andaluz de Biologia Molecular y Medicina Regenerativa - España |
| 3 | Recio, J. S. | Hombre |
Hospital de Salamanca - España
Instituto de Neurociencias de Castilla y León - España Univ Salamanca - España |
| 4 | Magdalena Martinez-Losa, Maria | Mujer |
CSIC - España
CSIC-JA-UPO-USE - Centro Andaluz de Biologia Molecular y Medicina Regenerativa - España |
| 4 | Martínez-Losa, María Magdalena | Mujer |
Consejo Superior de Investigaciones Científicas - España
CSIC-JA-UPO-USE - Centro Andaluz de Biologia Molecular y Medicina Regenerativa - España |
| 5 | ALONSO-PENA, JOSE RAMON | Hombre |
Hospital de Salamanca - España
IBSAL - España Universidad de Tarapacá - Chile Instituto de Neurociencias de Castilla y León - España Instituto de Investigación Biomédica de Salamanca (IBSAL) - España Univ Salamanca - España |
| 6 | Weruaga, Eduardo | Hombre |
Hospital de Salamanca - España
IBSAL - España Instituto de Neurociencias de Castilla y León - España Instituto de Investigación Biomédica de Salamanca (IBSAL) - España Univ Salamanca - España |
| 7 | Alvarez-Dolado, Manuel | Hombre |
Universidad de Tarapacá - Chile
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| Fuente |
|---|
| Ministerio de Economía y Competitividad |
| Ministerio de Ciencia e Innovación |
| Junta de Andalucía |
| Generalitat Valenciana |
| Fundacion Alicia Koplowitz |
| Junta de Castilla y Leon |
| Alicia Koplowitz Foundation |
| University of Salamanca |
| Fondo de Investigaciones Sanitarias |
| Consejeria de Educacion, Junta de Castilla y Leon |
| Military Manpower Administration |
| Centre for Regenerative Medicine and Cell Therapy of Castilla y Leon |
| Samuel Solorzano Foundation |
| FOLTRA Foundation |
| Mututa Madrilena (MMA) Foundation |
| Colectivo Ataxias en Movimiento Foundation |
| Harriet H. Samuelsson Foundation |
| Mututa Madrileña |
| Junta de Castilla y León, the Junta de Andalucía |
| Agradecimiento |
|---|
| Fondo de Investigaciones Sanitarias, Grant/Award Number: FIS 04/2744; Ministerio de Ciencia e Innovacion, Grant/Award Number: BFU2010-18284; Ministerio de Economia y Competitividad, Grant/Award Number: SAF2013-41175R; Generalitat Valenciana, Grant/Award Number: ACOMP06/131; Junta de Castilla y Leon; Junta de Andalucia, Grant/Award Number: PI-0736-2010; Centre for Regenerative Medicine and Cell Therapy of Castilla y Leon; Samuel Solorzano Foundation; FOLTRA Foundation; Mututa Madrilena (MMA) Foundation; Colectivo Ataxias en Movimiento Foundation; Alicia Koplowitz Foundation; University of Salamanca |
| This work was supported by the Fondo de Investigaciones Sanitarias (FIS 04/2744), the Ministerio de Ciencia e Innovación (BFU2010‐ 18284), the Ministerio de Economía y Competitividad (SAF2013‐ 41175R), the Generalitat Valenciana (ACOMP06/131), the Junta de Castilla y León, the Junta de Andalucía (PI‐0736‐2010), the Centre for Regenerative Medicine and Cell Therapy of Castilla y León, the Samuel Solórzano Foundation, the FOLTRA Foundation, the Mututa Madrileña (MMA) Foundation, the Colectivo Ataxias en Movimiento Foundation, the Alicia Koplowitz Foundation, and the University of Salamanca. |