A marginal level of dystrophin partially ameliorates hindlimb muscle passive mechanical properties in dystrophin-null mice

Muscle and Nerve

Volumn 46, Issue 6, 2012, Pages 943-947

  Hakim, Chady H ^a   Duan, Dongsheng ^a  

^a University of Missouri   (United States)

Author keywords

Dystrophin; EDL muscle; Mdx3cv mice; Mdx4cv mice; Passive properties

Indexed keywords

DYSTROPHIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; DUCHENNE MUSCULAR DYSTROPHY; EXTENSOR DIGITORUM LONGUS MUSCLE; IN VITRO STUDY; MALE; MOUSE; MUSCLE MASS; MUSCLE RIGIDITY; MYOFIBROSIS; NONHUMAN; PRIORITY JOURNAL; PROTEIN EXPRESSION; VISCOSITY;

EID: 84870215284     PISSN: 0148639X     EISSN: 10974598     Source Type: Journal    
DOI: 10.1002/mus.23536     Document Type: Article

References (13)

1. Cornu C, Goubel F, Fardeau M. Stiffness of knee extensors in Duchenne muscular dystrophy. Muscle Nerve 1998; 21: 1772-1774.
2. Cornu C, Goubel F, Fardeau M. Muscle and joint elastic properties during elbow flexion in Duchenne muscular dystrophy. J Physiol 2001; 533: 605-616.
3. Hakim CH, Grange RW, Duan D. The passive mechanical properties of the extensor digitorum longus muscle are compromised in 2 to 20-month-old mdx mice. J Appl Physiol 2011; 110: 1656-1663.
4. Hakim CH, Duan D. Gender differences in contractile and passive properties of mdx extensor digitorum longus muscle. Muscle Nerve 2012; 45: 250-256.
5. Chapman VM, Miller DR, Armstrong D, Caskey CT. Recovery of induced mutations for X chromosome-linked muscular dystrophy in mice. Proc Natl Acad Sci USA 1989; 86: 1292-1296.
6. Cox GA, Phelps SF, Chapman VM, Chamberlain JS. New mdx mutation disrupts expression of muscle and nonmuscle isoforms of dystrophin. Nat Genet 1993; 4: 87-93.
7. Im WB, Phelps SF, Copen EH, Adams EG, Slightom JL, Chamberlain JS. Differential expression of dystrophin isoforms in strains of mdx mice with different mutations. Hum Mol Genet 1996; 5: 1149-1153.
8. Li D, Yue Y, Duan D. Preservation of muscle force in mdx3cv mice correlates with low-level expression of a near full-length dystrophin protein. Am J Pathol 2008; 172: 1332-1341.
9. Bulman DE, Murphy EG, Zubrzycka-Gaarn EE, Worton RG, Ray PN. Differentiation of Duchenne and Becker muscular dystrophy phenotypes with amino- and carboxy-terminal antisera specific for dystrophin. Am J Hum Genet 1991; 48: 295-304.
10. Hoffman EP, Kunkel LM, Angelini C, Clarke A, Johnson M, Harris JB. Improved diagnosis of Becker muscular dystrophy by dystrophin testing. Neurology 1989; 39: 1011-1017.
11. Phelps SF, Hauser MA, Cole NM, Rafael JA, Hinkle RT, Faulkner JA, et al. Expression of full-length and truncated dystrophin mini-genes in transgenic mdx mice. Hum Mol Genet 1995; 4: 1251-1258.
12. Wells DJ, Wells KE, Walsh FS, Davies KE, Goldspink G, Love DR, et al. Human dystrophin expression corrects the myopathic phenotype in transgenic mdx mice. Hum Mol Genet 1992; 1: 35-40.
13. Li D, Yue Y, Duan D. Marginal level dystrophin expression improves clinical outcome in a strain of dystrophin/utrophin double knockout mice. PLoS One 2010; 5( 12): e15286.