Metabolic profiles of dystrophin and utrophin expression in mouse models of Duchenne muscular dystrophy

被引:31
作者
Griffin, JL [1 ]
Sang, E
Evens, T
Davies, K
Clarke, K
机构
[1] Univ London Imperial Coll Sci Technol & Med, Fac Med, London SW7 2AZ, England
[2] Univ Oxford, Dept Biochem, Oxford OX1 3QU, England
[3] Univ Oxford, Dept Human Anat & Genet, Oxford OX1 3QU, England
关键词
dystrophin; utrophin; Duchenne muscular dystrophy; metabolomics; metabolic profile;
D O I
10.1016/S0014-5793(02)03437-3
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Metabolic profiles from H-1 nuclear magnetic resonance spectroscopy have been used to describe both one and two protein systems in four mouse models related to Duchenne muscular dystrophy using the pattern recognition technique partial least squares. Robust statistical models were built for extracts and intact cardiac tissue, distinguishing mice according to expression of dystrophin. Using metabolic profiles of diaphragm, models were built describing dystrophin and utrophin, a dystrophin related protein, expression. Increased utrophin expression counteracted some of the deficits associated with dystrophic tissue. This suggests the method may be ideal for following treatment regimes such as gene therapy. (C) 2002 Federation of European Biochemical Societies. Published by Elsevier Science B.V. All rights reserved.
引用
收藏
页码:109 / 116
页数:8
相关论文
共 41 条
[1]   THE STRUCTURAL AND FUNCTIONAL DIVERSITY OF DYSTROPHIN [J].
AHN, AH ;
KUNKEL, LM .
NATURE GENETICS, 1993, 3 (04) :283-291
[2]   Aminoglycoside antibiotics restore dystrophin function to skeletal muscles of mdx mice [J].
Barton-Davis, ER ;
Cordier, L ;
Shoturma, DI ;
Leland, SE ;
Sweeney, HL .
JOURNAL OF CLINICAL INVESTIGATION, 1999, 104 (04) :375-381
[3]   A second promoter provides an alternative target for therapeutic up-regulation of utrophin in Duchenne muscular dystrophy [J].
Burton, EA ;
Tinsley, JM ;
Holzfeind, PJ ;
Rodrigues, NR ;
Davies, KE .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1999, 96 (24) :14025-14030
[4]   Muscular dystrophy - Reason for optimism? [J].
Burton, EA ;
Davies, KE .
CELL, 2002, 108 (01) :5-8
[5]   Rescue of skeletal muscles of γ-sarcoglycan-deficient mice with adeno-associated virus-mediated gene transfer [J].
Cordier, L ;
Hack, AA ;
Scott, MO ;
Barton-Davis, ER ;
Gao, GP ;
Wilson, JM ;
McNally, EM ;
Sweeney, HL .
MOLECULAR THERAPY, 2000, 1 (02) :119-129
[6]   Expression of truncated utrophin leads to major functional improvements in dystrophin-deficient muscles of mice [J].
Deconinck, N ;
Tinsley, J ;
DeBacker, F ;
Fisher, R ;
Kahn, D ;
Phelps, S ;
Davies, K ;
Gillis, JM .
NATURE MEDICINE, 1997, 3 (11) :1216-1221
[7]   Molecular and functional analysis of the utrophin promoter [J].
Dennis, CL ;
Tinsley, JM ;
Deconinck, AE ;
Davies, KE .
NUCLEIC ACIDS RESEARCH, 1996, 24 (09) :1646-1652
[8]   Investigation of skeletal muscle denervation and reinnervation using magnetic resonance spectroscopy [J].
Dort, JC ;
Fan, Y ;
McIntyre, DD .
OTOLARYNGOLOGY-HEAD AND NECK SURGERY, 2001, 125 (06) :617-622
[9]  
Emery A E, 1991, Neuromuscul Disord, V1, P19, DOI 10.1016/0960-8966(91)90039-U
[10]  
Eriksson L., 1999, INTRO MULTI MEGAVARI