Endpoint measures in the mdx mouse relevant for muscular dystrophy pre-clinical studies

Yvonne M Kobayashi; Erik P Rader; Robert W Crawford; Kevin P Campbell

doi:10.1016/j.nmd.2011.08.001

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Endpoint measures in the mdx mouse relevant for muscular dystrophy pre-clinical studies

Journal article

Peer reviewed

Endpoint measures in the mdx mouse relevant for muscular dystrophy pre-clinical studies

Yvonne M Kobayashi, Erik P Rader, Robert W Crawford and Kevin P Campbell

Neuromuscular disorders : NMD, Vol.22(1), pp.34-42

01/2012

DOI: 10.1016/j.nmd.2011.08.001

PMCID: PMC3264796

PMID: 22154712

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https://www.ncbi.nlm.nih.gov/pmc/articles/3264796View

Open Access

Abstract

Loss of mobility influences the quality of life for patients with neuromuscular diseases. Common measures of mobility and chronic muscle damage are the six-minute walk test and serum creatine kinase. Despite extensive pre-clinical studies of therapeutic approaches, characterization of these measures is incomplete. To address this, a six-minute ambulation assay, serum creatine kinase, and myoglobinuria were investigated for the mdx mouse, a dystrophinopathy mouse model commonly used in pre-clinical studies. mdx mice ambulated shorter distances than normal controls, a disparity accentuated after mild exercise. An asymmetric pathophysiology in mdx mice was unmasked with exercise, and peak measurements of serum creatine kinase and myoglobinuria were identified. Our data highlights the necessity to consider asymmetric pathology and timing of biomarkers when testing potential therapies for muscular dystrophy.

Creatine Kinase - blood

Mice, Inbred C57BL

Male

Muscular Dystrophy, Duchenne - pathology

Physical Conditioning, Animal - physiology

Animals

Muscular Dystrophy, Duchenne - physiopathology

Muscle, Skeletal - physiopathology

Myoglobinuria - urine

Mice, Inbred mdx

Mice

Muscle, Skeletal - pathology

Disease Models, Animal

Muscular Dystrophy, Duchenne - diagnosis

Details

Title: Subtitle: Endpoint measures in the mdx mouse relevant for muscular dystrophy pre-clinical studies
Creators: Yvonne M Kobayashi - Department of Molecular Physiology and Biophysics, Howard Hughes Medical Institute, University of Iowa Roy J. and Lucille A. Carver College of Medicine, City, IA 52242-1101, USA
Erik P Rader
Robert W Crawford
Kevin P Campbell
Resource Type: Journal article
Publication Details: Neuromuscular disorders : NMD, Vol.22(1), pp.34-42
DOI: 10.1016/j.nmd.2011.08.001
PMID: 22154712
PMCID: PMC3264796
NLM abbreviation: Neuromuscul Disord
ISSN: 0960-8966
eISSN: 1873-2364
Publisher: England
Grant note: F32 AR048742 / NIAMS NIH HHS T32 HL07121 / NHLBI NIH HHS R01-AR051199 / NIAMS NIH HHS Howard Hughes Medical Institute R01-AR051199-S / NIAMS NIH HHS F32-AR48742 / NIAMS NIH HHS R01 AR051199 / NIAMS NIH HHS U54 NS053672-08 / NINDS NIH HHS U54 NS053672 / NINDS NIH HHS U54-NS053672 / NINDS NIH HHS T32 HL007121 / NHLBI NIH HHS UL1-RR0024979 / NCRR NIH HHS UL1 RR024979 / NCRR NIH HHS U54-NS053672-S / NINDS NIH HHS
Language: English
Date published: 01/2012
Academic Unit: Neurology; Molecular Physiology and Biophysics; Iowa Neuroscience Institute
Record Identifier: 9984020866702771

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