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A Family of Laminin α2 Chain-Deficient Mouse Mutants: Advancing the Research on LAMA2-CMD

The research on laminin α2 chain-deficient congenital muscular dystrophy (LAMA2-CMD) advanced rapidly in the last few decades, largely due to availability of good mouse models for the disease and a strong interest in preclinical studies from scientists all over the world. These mouse models continue...

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Detalles Bibliográficos
Autores principales: Gawlik, Kinga I., Durbeej, Madeleine
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7188397/
https://www.ncbi.nlm.nih.gov/pubmed/32457577
http://dx.doi.org/10.3389/fnmol.2020.00059
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author Gawlik, Kinga I.
Durbeej, Madeleine
author_facet Gawlik, Kinga I.
Durbeej, Madeleine
author_sort Gawlik, Kinga I.
collection PubMed
description The research on laminin α2 chain-deficient congenital muscular dystrophy (LAMA2-CMD) advanced rapidly in the last few decades, largely due to availability of good mouse models for the disease and a strong interest in preclinical studies from scientists all over the world. These mouse models continue to provide a solid platform for understanding the LAMA2-CMD pathology. In addition, they enable researchers to test laborious, necessary routines, but also the most creative scientific approaches in order to design therapy for this devastating disorder. In this review we present animals belonging to the laminin α2 chain-deficient “dy/dy” mouse family (dy/dy, dy(2J)/dy(2J), dy(3K)/dy(3K), dy(W)/dy(W), et al.) and a summary of the scientific progress they facilitated. We also raise a few questions that need to be addressed in order to maximize the usefulness of laminin α2 murine mutants and to further advance the LAMA2-CMD studies. We believe that research opportunities offered by the mouse models for LAMA2-CMD will continuously support our efforts to find a treatment for the disease.
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spelling pubmed-71883972020-05-25 A Family of Laminin α2 Chain-Deficient Mouse Mutants: Advancing the Research on LAMA2-CMD Gawlik, Kinga I. Durbeej, Madeleine Front Mol Neurosci Neuroscience The research on laminin α2 chain-deficient congenital muscular dystrophy (LAMA2-CMD) advanced rapidly in the last few decades, largely due to availability of good mouse models for the disease and a strong interest in preclinical studies from scientists all over the world. These mouse models continue to provide a solid platform for understanding the LAMA2-CMD pathology. In addition, they enable researchers to test laborious, necessary routines, but also the most creative scientific approaches in order to design therapy for this devastating disorder. In this review we present animals belonging to the laminin α2 chain-deficient “dy/dy” mouse family (dy/dy, dy(2J)/dy(2J), dy(3K)/dy(3K), dy(W)/dy(W), et al.) and a summary of the scientific progress they facilitated. We also raise a few questions that need to be addressed in order to maximize the usefulness of laminin α2 murine mutants and to further advance the LAMA2-CMD studies. We believe that research opportunities offered by the mouse models for LAMA2-CMD will continuously support our efforts to find a treatment for the disease. Frontiers Media S.A. 2020-04-21 /pmc/articles/PMC7188397/ /pubmed/32457577 http://dx.doi.org/10.3389/fnmol.2020.00059 Text en Copyright © 2020 Gawlik and Durbeej. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neuroscience
Gawlik, Kinga I.
Durbeej, Madeleine
A Family of Laminin α2 Chain-Deficient Mouse Mutants: Advancing the Research on LAMA2-CMD
title A Family of Laminin α2 Chain-Deficient Mouse Mutants: Advancing the Research on LAMA2-CMD
title_full A Family of Laminin α2 Chain-Deficient Mouse Mutants: Advancing the Research on LAMA2-CMD
title_fullStr A Family of Laminin α2 Chain-Deficient Mouse Mutants: Advancing the Research on LAMA2-CMD
title_full_unstemmed A Family of Laminin α2 Chain-Deficient Mouse Mutants: Advancing the Research on LAMA2-CMD
title_short A Family of Laminin α2 Chain-Deficient Mouse Mutants: Advancing the Research on LAMA2-CMD
title_sort family of laminin α2 chain-deficient mouse mutants: advancing the research on lama2-cmd
topic Neuroscience
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7188397/
https://www.ncbi.nlm.nih.gov/pubmed/32457577
http://dx.doi.org/10.3389/fnmol.2020.00059
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