Comparison of skeletal muscle pathology and motor function of dystrophin and utrophin deficient mouse strains.

M. van Putten; D. Kumar; M. Hulsker; W.M.H. Hoogaars; J.J. Plomp; A. van Opstal; M. van Iterson; P. Admiraal; G.J. van Ommen; P.A. 't Hoen; A. Aartsma-Rus

doi:10.1016/j.nmd.2011.10.011

Comparison of skeletal muscle pathology and motor function of dystrophin and utrophin deficient mouse strains.

M. van Putten, D. Kumar, M. Hulsker, W.M.H. Hoogaars, J.J. Plomp, A. van Opstal, M. van Iterson, P. Admiraal, G.J. van Ommen, P.A. 't Hoen, A. Aartsma-Rus

Research output: Contribution to Journal › Article › Academic › peer-review

Abstract

Abstract
The genetic defect of mdx mice resembles that of Duchenne muscular dystrophy, although their functional performance and life expectancy is nearly normal. By contrast, mice lacking utrophin and dystrophin (mdx/utrn -/-) are severely affected and die prematurely. Mice with one utrophin allele (mdx/utrn +/-) are more severely affected than mdx mice, but outlive mdx/utrn -/- mice. We subjected mdx/utrn +/+, +/-, -/- and wild type males to a 12week functional test regime of four different functional tests. Mdx/utrn +/+ and +/- mice completed the regime, while mdx/utrn -/- mice died prematurely. Mdx/utrn +/- mice performed significantly worse compared to mdx/utrn +/+ mice in functional tests. Creatine kinase levels, percentage of fibrotic/necrotic tissue, morphology of neuromuscular synapses and expression of biomarker genes were comparable, whereas mdx/utrn +/- and -/- mice had increased levels of regenerating fibers. This makes mdx/utrn +/- mice valuable for testing the benefit of potential therapies on muscle function parameters.

Original language	English
Pages (from-to)	406-417
Journal	Neuromuscular Disorders
Volume	22
Issue number	5
DOIs	https://doi.org/10.1016/j.nmd.2011.10.011
Publication status	Published - May 2012
Externally published	Yes

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van Putten, M., Kumar, D., Hulsker, M., Hoogaars, W. M. H., Plomp, J. J., van Opstal, A., van Iterson, M., Admiraal, P., van Ommen, G. J., 't Hoen, P. A., & Aartsma-Rus, A. (2012). Comparison of skeletal muscle pathology and motor function of dystrophin and utrophin deficient mouse strains. Neuromuscular Disorders, 22(5), 406-417. https://doi.org/10.1016/j.nmd.2011.10.011

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title = "Comparison of skeletal muscle pathology and motor function of dystrophin and utrophin deficient mouse strains.",

abstract = "AbstractThe genetic defect of mdx mice resembles that of Duchenne muscular dystrophy, although their functional performance and life expectancy is nearly normal. By contrast, mice lacking utrophin and dystrophin (mdx/utrn -/-) are severely affected and die prematurely. Mice with one utrophin allele (mdx/utrn +/-) are more severely affected than mdx mice, but outlive mdx/utrn -/- mice. We subjected mdx/utrn +/+, +/-, -/- and wild type males to a 12week functional test regime of four different functional tests. Mdx/utrn +/+ and +/- mice completed the regime, while mdx/utrn -/- mice died prematurely. Mdx/utrn +/- mice performed significantly worse compared to mdx/utrn +/+ mice in functional tests. Creatine kinase levels, percentage of fibrotic/necrotic tissue, morphology of neuromuscular synapses and expression of biomarker genes were comparable, whereas mdx/utrn +/- and -/- mice had increased levels of regenerating fibers. This makes mdx/utrn +/- mice valuable for testing the benefit of potential therapies on muscle function parameters.",

author = "{van Putten}, M. and D. Kumar and M. Hulsker and W.M.H. Hoogaars and J.J. Plomp and {van Opstal}, A. and {van Iterson}, M. and P. Admiraal and {van Ommen}, G.J. and {'t Hoen}, P.A. and A. Aartsma-Rus",

year = "2012",

month = may,

doi = "10.1016/j.nmd.2011.10.011",

language = "English",

volume = "22",

pages = "406--417",

journal = "Neuromuscular Disorders",

issn = "0960-8966",

publisher = "Elsevier Limited",

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TY - JOUR

T1 - Comparison of skeletal muscle pathology and motor function of dystrophin and utrophin deficient mouse strains.

AU - van Putten, M.

AU - Kumar, D.

AU - Hulsker, M.

AU - Hoogaars, W.M.H.

AU - Plomp, J.J.

AU - van Opstal, A.

AU - van Iterson, M.

AU - Admiraal, P.

AU - van Ommen, G.J.

AU - 't Hoen, P.A.

AU - Aartsma-Rus, A.

PY - 2012/5

Y1 - 2012/5

N2 - AbstractThe genetic defect of mdx mice resembles that of Duchenne muscular dystrophy, although their functional performance and life expectancy is nearly normal. By contrast, mice lacking utrophin and dystrophin (mdx/utrn -/-) are severely affected and die prematurely. Mice with one utrophin allele (mdx/utrn +/-) are more severely affected than mdx mice, but outlive mdx/utrn -/- mice. We subjected mdx/utrn +/+, +/-, -/- and wild type males to a 12week functional test regime of four different functional tests. Mdx/utrn +/+ and +/- mice completed the regime, while mdx/utrn -/- mice died prematurely. Mdx/utrn +/- mice performed significantly worse compared to mdx/utrn +/+ mice in functional tests. Creatine kinase levels, percentage of fibrotic/necrotic tissue, morphology of neuromuscular synapses and expression of biomarker genes were comparable, whereas mdx/utrn +/- and -/- mice had increased levels of regenerating fibers. This makes mdx/utrn +/- mice valuable for testing the benefit of potential therapies on muscle function parameters.

AB - AbstractThe genetic defect of mdx mice resembles that of Duchenne muscular dystrophy, although their functional performance and life expectancy is nearly normal. By contrast, mice lacking utrophin and dystrophin (mdx/utrn -/-) are severely affected and die prematurely. Mice with one utrophin allele (mdx/utrn +/-) are more severely affected than mdx mice, but outlive mdx/utrn -/- mice. We subjected mdx/utrn +/+, +/-, -/- and wild type males to a 12week functional test regime of four different functional tests. Mdx/utrn +/+ and +/- mice completed the regime, while mdx/utrn -/- mice died prematurely. Mdx/utrn +/- mice performed significantly worse compared to mdx/utrn +/+ mice in functional tests. Creatine kinase levels, percentage of fibrotic/necrotic tissue, morphology of neuromuscular synapses and expression of biomarker genes were comparable, whereas mdx/utrn +/- and -/- mice had increased levels of regenerating fibers. This makes mdx/utrn +/- mice valuable for testing the benefit of potential therapies on muscle function parameters.

U2 - 10.1016/j.nmd.2011.10.011

DO - 10.1016/j.nmd.2011.10.011

M3 - Article

SN - 0960-8966

VL - 22

SP - 406

EP - 417

JO - Neuromuscular Disorders

JF - Neuromuscular Disorders

IS - 5

ER -

Comparison of skeletal muscle pathology and motor function of dystrophin and utrophin deficient mouse strains.

Abstract

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