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Intramuscular transplantation of bone marrow cells prolongs the lifespan of SOD1(G93A) mice and modulates expression of prognosis biomarkers of the disease
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive muscle weakness, paralysis and death. There is no effective treatment for ALS and stem cell therapy has arisen as a potential therapeutic approach. METHODS: SOD1 mutant mice were used to stud...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5889612/ https://www.ncbi.nlm.nih.gov/pubmed/29625589 http://dx.doi.org/10.1186/s13287-018-0843-z |
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author | Rando, Amaya Pastor, Diego Viso-León, Mari Carmen Martínez, Anna Manzano, Raquel Navarro, Xavier Osta, Rosario Martínez, Salvador |
author_facet | Rando, Amaya Pastor, Diego Viso-León, Mari Carmen Martínez, Anna Manzano, Raquel Navarro, Xavier Osta, Rosario Martínez, Salvador |
author_sort | Rando, Amaya |
collection | PubMed |
description | BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive muscle weakness, paralysis and death. There is no effective treatment for ALS and stem cell therapy has arisen as a potential therapeutic approach. METHODS: SOD1 mutant mice were used to study the potential neurotrophic effect of bone marrow cells grafted into quadriceps femoris muscle. RESULTS: Bone marrow intramuscular transplants resulted in increased longevity with improved motor function and decreased motoneuron degeneration in the spinal cord. Moreover, the increment of the glial-derived neurotrophic factor and neurotrophin 4 observed in the grafted muscles suggests that this partial neuroprotective effect is mediated by neurotrophic factor release at the neuromuscular junction level. Finally, certain neurodegeneration and muscle disease-specific markers, which are altered in the SOD1(G93A) mutant mouse and may serve as molecular biomarkers for the early detection of ALS in patients, have been studied with encouraging results. CONCLUSIONS: This work demonstrates that stem cell transplantation in the muscle prolonged the lifespan, increased motoneuron survival and slowed disease progression, which was also assessed by genetic expression analysis. |
format | Online Article Text |
id | pubmed-5889612 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-58896122018-04-10 Intramuscular transplantation of bone marrow cells prolongs the lifespan of SOD1(G93A) mice and modulates expression of prognosis biomarkers of the disease Rando, Amaya Pastor, Diego Viso-León, Mari Carmen Martínez, Anna Manzano, Raquel Navarro, Xavier Osta, Rosario Martínez, Salvador Stem Cell Res Ther Research BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive muscle weakness, paralysis and death. There is no effective treatment for ALS and stem cell therapy has arisen as a potential therapeutic approach. METHODS: SOD1 mutant mice were used to study the potential neurotrophic effect of bone marrow cells grafted into quadriceps femoris muscle. RESULTS: Bone marrow intramuscular transplants resulted in increased longevity with improved motor function and decreased motoneuron degeneration in the spinal cord. Moreover, the increment of the glial-derived neurotrophic factor and neurotrophin 4 observed in the grafted muscles suggests that this partial neuroprotective effect is mediated by neurotrophic factor release at the neuromuscular junction level. Finally, certain neurodegeneration and muscle disease-specific markers, which are altered in the SOD1(G93A) mutant mouse and may serve as molecular biomarkers for the early detection of ALS in patients, have been studied with encouraging results. CONCLUSIONS: This work demonstrates that stem cell transplantation in the muscle prolonged the lifespan, increased motoneuron survival and slowed disease progression, which was also assessed by genetic expression analysis. BioMed Central 2018-04-06 /pmc/articles/PMC5889612/ /pubmed/29625589 http://dx.doi.org/10.1186/s13287-018-0843-z Text en © The Author(s). 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Research Rando, Amaya Pastor, Diego Viso-León, Mari Carmen Martínez, Anna Manzano, Raquel Navarro, Xavier Osta, Rosario Martínez, Salvador Intramuscular transplantation of bone marrow cells prolongs the lifespan of SOD1(G93A) mice and modulates expression of prognosis biomarkers of the disease |
title | Intramuscular transplantation of bone marrow cells prolongs the lifespan of SOD1(G93A) mice and modulates expression of prognosis biomarkers of the disease |
title_full | Intramuscular transplantation of bone marrow cells prolongs the lifespan of SOD1(G93A) mice and modulates expression of prognosis biomarkers of the disease |
title_fullStr | Intramuscular transplantation of bone marrow cells prolongs the lifespan of SOD1(G93A) mice and modulates expression of prognosis biomarkers of the disease |
title_full_unstemmed | Intramuscular transplantation of bone marrow cells prolongs the lifespan of SOD1(G93A) mice and modulates expression of prognosis biomarkers of the disease |
title_short | Intramuscular transplantation of bone marrow cells prolongs the lifespan of SOD1(G93A) mice and modulates expression of prognosis biomarkers of the disease |
title_sort | intramuscular transplantation of bone marrow cells prolongs the lifespan of sod1(g93a) mice and modulates expression of prognosis biomarkers of the disease |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5889612/ https://www.ncbi.nlm.nih.gov/pubmed/29625589 http://dx.doi.org/10.1186/s13287-018-0843-z |
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