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

Gawlik, Kinga I. LU and Durbeej, Madeleine LU (2020) In Frontiers in Molecular Neuroscience 13.
Abstract

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, dy2J/dy2J, dy3K/dy3K,... (More)

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, dy2J/dy2J, dy3K/dy3K, dyW/dyW, 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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author
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organization
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type
Contribution to journal
publication status
published
subject
keywords
animal model, basement membrane, knockout, laminin, muscular dystrophy, transgene
in
Frontiers in Molecular Neuroscience
volume
13
article number
59
publisher
Frontiers Media S. A.
external identifiers
  • scopus:85085622342
  • pmid:32457577
ISSN
1662-5099
DOI
10.3389/fnmol.2020.00059
language
English
LU publication?
yes
id
f42623dd-a49e-4949-953b-4aab5f14612e
date added to LUP
2020-06-24 11:44:06
date last changed
2024-03-04 21:45:44
@article{f42623dd-a49e-4949-953b-4aab5f14612e,
  abstract     = {{<p>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<sup>2J</sup>/dy<sup>2J</sup>, dy<sup>3K</sup>/dy<sup>3K</sup>, dy<sup>W</sup>/dy<sup>W</sup>, 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.</p>}},
  author       = {{Gawlik, Kinga I. and Durbeej, Madeleine}},
  issn         = {{1662-5099}},
  keywords     = {{animal model; basement membrane; knockout; laminin; muscular dystrophy; transgene}},
  language     = {{eng}},
  month        = {{04}},
  publisher    = {{Frontiers Media S. A.}},
  series       = {{Frontiers in Molecular Neuroscience}},
  title        = {{A Family of Laminin α2 Chain-Deficient Mouse Mutants : Advancing the Research on LAMA2-CMD}},
  url          = {{http://dx.doi.org/10.3389/fnmol.2020.00059}},
  doi          = {{10.3389/fnmol.2020.00059}},
  volume       = {{13}},
  year         = {{2020}},
}