Description

Strain Information

Type Coisogenic; Mutant Strain; Spontaneous Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Mating System Homozygote x Hemizygote         (Female x Male) Species laboratory mouse H2 Haplotype b Generation F66 (03-JAN-08)

Appearance
black, affected
Related Genotype: a/a Dmd^mdx/Dmd^mdx or a/a Dmd^mdx/Y

Description
The X-linked dystrophin gene (Dmd) is highly expressed in muscle cells and encodes a cytoskeletal protein which localizes to the inner face of the sarcolemma. Dystrophin molecules bind to cytoskeletal F-actin and transmembrane beta-dystroglycan as part of a complex, multimolecular unit that mediates signaling between the intracellular cytoskeleton and the extracellular matrix. The structure and localization also suggest that dystrophin is important for stabilizing the plasma membrane, particularly during contraction. The mdx mutation of Dmd is recessive and heterozygous females are visually indistinguishable from wild-type mice. Females homozygous and males hemizygous for the Dmd^mdx allele retain a normal lifespan and can survive up to two years. Like human patients who suffer from one of the most common neuromuscular diseases, Duchenne muscular dystrophy (DMD), the Dmd^mdx mutants do not express dystrophin and therefore have been routinely used as an animal model of the disease even though the resultant myopathology is much less severe compared to the human disease course.

Muscle from Dmd^mdx mutants is histologically normal early in postnatal development, but starting around 3 weeks muscle necrosis develops with some visible muscle weakness. Biochemical analysis of related pathologies includes elevated serum creatine kinase and pyruvate kinase levels, along with an accumulation of macrophages, both early markers of muscle degeneration. While skeletal limb muscles are characterized by a persistent and progressive degeneration and necrosis, this is offset by a regenerative response activated by satellite cells and muscle hypertrophy. The regenerating fibers are morphologically typified by small-caliber centrally nucleated fibers; nevertheless, the mice assume normal behavior. The muscles of Dmd^mdx mutants have an overall reduction in elasticity, making them more susceptible to injury due to lengthening-activation. Interestingly, the mutant leg muscles were found to initially develop normally, but the differentiation of regenerated myotubes into both fast and slow fiber types was significantly inhibited. The comparatively mild phenotype of the Dmd^mdx mice can, in part, be attributed to the compensatory function of the dystrophin-related protein utrophin, which is highly upregulated in regenerating muscle fibers in adult Dmd^mdx mutants. This functional redundancy was demonstrated in mice deficient for both of these sarcolemmal proteins where the observed muscular dystrophy was much more severe and led to a premature death in the dystophin/utrophin double mutants. Also, the muscle-specific transcription factor MYOD may also be involved in facilitating muscle regeneration in the mutant mice as Dmd^mdx mice also lacking MYOD exhibit a more severe dystrophy of the muscles. In contrast to limb muscles, the diaphragm muscles of Dmd^mdx mice do not undergo a significant regeneration phase such that the continuous dystrophy weakens these muscles with age. The specific twitch force, specific titanic force and maximum power are all reduced in the diaphragm of Dmd^mdx mutants.

Auditory function of Dmd^mdx mutants, as assessed by brainstem auditory evoked potentials, is altered leaving them more vulnerable to noise damage. In mouse cardiac myocytes, dystrophin colocalizes with L-type calcium channels; in Dmd^mdx mutants, the inactivation of these channels is reduced and voltage-dependant activation shifts to more positive potentials, providing evidence that the protein normally regulates calcium channel activity in cardiac tissue. In brain areas associated with learning, memory and cognitive tasks, dystrophin and its isoforms have been detected within postsynaptic specializations. In the Dmd^mdx mouse sympathetic superior cervical ganglion, postsynaptic nicotinic acetylcholine receptor complexes containing the alpha3 subtype are destabilized as assayed by immunocytochemical and immunoprecipitation techniques. That proper dystrophin function is linked in nervous tissue to synaptic ligand-gated ion channel organization raises intriguing possibilities regarding the pathologic mechanisms underlying the cognitive defects often seen in DMD patients. (reviewed by Watchko et al. 2002, Durbeej and Campbell 2002; Ahn and Kunkel 1993; Cook and Davisson 1991; Doolittle 1997; Monaco and Kunkel 1987; Tamura et al. 1993; Stevens and Faulkner 2000; Del Signore et al. 2002; Houzelstein et al. 1992; Sicinski et al. 1989; Deconinck et al. 1997; Grady et al. 1997; Earnshaw et al. 2002; Chen et al. 2002; D'Souza et al. 1995; Lynch et al. 2001; Carretta et al. 2001; Sadeghi et al. 2002; Lidov et al. 1995)

Nuclear opacity (cataracts) can be seen in the lens of one day old mice. A slight anterior subcapsular opacity is seen by four days progressing to complete anterior subcapsular opacity in 150 day old mice.

Development
The spontaneous mutation Dmd^mdx arose just prior to 1977 at the Agricultural Research Council's Poultry Research Centre, U.K., in C57BL/10ScSn mice obtained from M. Festing (MRC Laboratory Animals Centre, Carshalton, Surrey, U.K.). Mice carrying the mdx allele were imported to The Jackson Laboratory in 1984 by Dr. Thomas Roderick, who received them from Dr. Karen Moore (Department of Genetics, University of California, Berkley). C57BL/10ScSn-Dmd^mdx is maintained by sibling mating homozygous females with hemizygous males; mice can breed up to six months of age. In 2002, the strain had reached F49. (Bulfield et al., 1978 and 1984).

Control Information

Related Strains

Strains carrying other alleles of Dmd

002388	B6Ros.Cg-Dmdmdx-2Cv/J
002377	B6Ros.Cg-Dmdmdx-3Cv/J
002378	B6Ros.Cg-Dmdmdx-4Cv/J
002379	B6Ros.Cg-Dmdmdx-5Cv/J

View Strains carrying other alleles of Dmd     (4 strains)

Additional Web Information

Genetic Quality Control Annual Report
JAX^® NOTES, Summer 1991; 446. C57BL/10ScSn-mdx/J.

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms

Muscular Dystrophy, Duchenne Type; DMD - Models with phenotypic similarity to human disease where etiologies involve orthologs.1

1 Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).

View Mammalian Phenotype Terms

Mammalian Phenotype Terms

      assigned by genotype

Dmd^mdx/Dmd^mdx

        C57BL/10ScSnJ

• muscle phenotype
• abnormal muscle morphology (MGI Ref ID J:7361)
• abnormal muscle fiber morphology (MGI Ref ID J:7361)
  • variable muscle fiber size; progressive starting at 3 weeks of age
  • development of electron-dense bodies in the mitochondria resulting in swelling and degenerating mitochondria, and disruption of the plasmalemma basal lamina
  • the normal myofibrillar architecture of bands and lines disappears and myofilaments disintegrate and become misaligned
• dilated sarcoplasmic reticulum (MGI Ref ID J:7361)
• muscle degeneration (MGI Ref ID J:7361)
  • progressive starting at 3 weeks of age
• myopathy (MGI Ref ID J:7361)
  • progressive degenerative myopathy; increased severity with age
• skeletal muscle necrosis (MGI Ref ID J:7361)
  • progressive starting at 9 weeks of age
• abnormal muscle physiology (MGI Ref ID J:3666)
  • increased intracellular sodium concentration in muscle; increased severity with age
• muscular atrophy (MGI Ref ID J:7361)
  • progressive starting at 3 weeks of age
• dystrophic muscle (MGI Ref ID J:7361)
• homeostasis/metabolism phenotype
• abnormal circulating enzyme level (MGI Ref ID J:7361)
  • exhibit elevated blood levels of muscle creatine kinase and pyruvate kinase
• reproductive system phenotype
• reduced female fertility (MGI Ref ID J:7361)
  • slight reduction in fertility
• other phenotype
• fibrosis (MGI Ref ID J:7361)
  • exhibit mild muscle fibrosis, however there is no replacement of lost muscle by fat cells

Dmd^mdx/Dmd^mdx

        C57BL/10ScSn-Dmd^mdx/J

• behavior/neurological phenotype
• abnormal grip strength (MGI Ref ID J:106640)
  • performance is similar to that observed in double knockouts
• impaired coordination (MGI Ref ID J:106640)
  • performance is similar to that observed in double knockouts
• hearing/vestibular/ear phenotype
• decreased brainstem auditory evoked potential (MGI Ref ID J:105938)
  • significantly increased hearing threshold and prolonged brainstem auditory evoked potential peak and interpeak latencies after noise exposure
• increased susceptibility to noise-induced hearing loss (MGI Ref ID J:105938)
  • daily exposure to noise for 1 month increased hearing threshold and prolonged brainstem auditory evoked potential peak and interpeak latencies

Dmd^mdx/Y

        C57BL/10ScSnJ

• behavior/neurological phenotype
• abnormal motor capabilities/coordination/movement (MGI Ref ID J:7361)
• impaired coordination (MGI Ref ID J:7361)
  • mild incoordination noticeable by 12 months of age
• tremors (MGI Ref ID J:7361)
  • muscular tremors noticeable by 12 months of age
• muscle phenotype
• abnormal muscle morphology (MGI Ref ID J:7361)
• abnormal muscle fiber morphology (MGI Ref ID J:7361)
  • variable muscle fiber size; progressive starting at 3 weeks of age
  • development of electron-dense bodies in the mitochondria resulting in swelling and degenerating mitochondria, and disruption of the plasmalemma basal lamina
  • the normal myofibrillar architecture of bands and lines disappears and myofilaments disintegrate and become misaligned
• dilated sarcoplasmic reticulum (MGI Ref ID J:7361)
• muscle degeneration (MGI Ref ID J:7361)
  • progressive starting at 3 weeks of age
• myopathy (MGI Ref ID J:7361)
  • progressive degenerative myopathy; increased severity with age
• skeletal muscle necrosis (MGI Ref ID J:7361)
  • progressive starting at 9 weeks of age
• abnormal muscle physiology (MGI Ref ID J:3666)
  • increased intracellular sodium concentration in muscle; increased severity with age
• muscular atrophy (MGI Ref ID J:7361)
  • progressive starting at 3 weeks of age
• dystrophic muscle (MGI Ref ID J:7361)
• homeostasis/metabolism phenotype
• abnormal circulating enzyme level (MGI Ref ID J:7361)
  • exhibit elevated blood levels of muscle creatine kinase and pyruvate kinase
• other phenotype
• fibrosis (MGI Ref ID J:7361)
  • exhibit mild muscle fibrosis, however there is no replacement of lost muscle by fat cells

Dmd^mdx/Y

        C57BL/10ScSn-Dmd^mdx

• muscle phenotype
• abnormal skeletal muscle fiber morphology (MGI Ref ID J:9638)
  • mice exhibit skeletal muscle fiber degeneration with phagocytosis unlike in wild-type mice
• impaired muscle relaxation (MGI Ref ID J:9638)
  • electromyograms reveal peudomyotonia unlike in wild-type mice
• muscle degeneration (MGI Ref ID J:9638)
  • mice exhibit skeletal muscle fiber degeneration with phagocytosis unlike in wild-type mice
• myocardial fiber degeneration (MGI Ref ID J:9638)
  • unlike in wild-type mice, cardiac fiber degeneration with phagocytosis is observed
• cardiovascular system phenotype
• myocardial fiber degeneration (MGI Ref ID J:9638)
  • unlike in wild-type mice, cardiac fiber degeneration with phagocytosis is observed

The following phenotype information may relate to a genetic background differing from this JAX^® Mice strain.

Dmd^mdx/Y

        B6.B10ScSn-Dmd^mdx

• muscle phenotype
• abnormal skeletal muscle morphology (MGI Ref ID J:85088)
  • muscles contain occasional patches of pale tissue which are primarily connective tissue
• abnormal skeletal muscle fiber morphology (MGI Ref ID J:85088)
  • when mice are injected with the diazo dye EBD, dye is taken up by damaged muscle fibers to varying degrees, but preferentially into the hindlimb; uptake occurs because of focal breakdown of plasmalemma which is an early event in necrosis
  • muscles are not stained homogeneously, but streaks of dye representing damaged muscle fibers are observed in the muscle fibers of the diaphragm and gluteus maximus; damage is less severe than in Fgf2/Fgf6/Dmd triple mutants
• dystrophic muscle (MGI Ref ID J:85088)
  • mutants show dystrophic changes to the muscle fibers, although less severely than Fgf2/Fgf6/Dmd triple mutants

View Research Applications

Research Applications

This mouse can be used to support research in many areas including:

Dmd^mdx related

Cell Biology Research
Signal Transduction

Mouse/Human Gene Homologs
muscular dystrophy (Duchenne and Becker)

Neurobiology Research
Neuromuscular Defects

Sensorineural Research
Cataracts

Genes & Alleles

Gene & Allele Information

Genotyping

Genotyping Information

Genotyping Protocols

Dmd^mdx, PYRO, vers. 1

Helpful Links

Optimizing PCR Protocols

References

References

Selected Reference(s)

Bulfield G; Siller WG; Wight PA; Moore KJ. 1984. X chromosome-linked muscular dystrophy (mdx) in the mouse. Proc Natl Acad Sci U S A 81(4):1189-92. [PubMed: 6583703]  [MGI Ref ID J:7361]

Ryder-Cook AS; Sicinski P; Thomas K; Davies KE; Worton RG; Barnard EA; Darlison MG; Barnard PJ. 1988. Localization of the mdx mutation within the mouse dystrophin gene. EMBO J 7(10):3017-21. [PubMed: 2903046]  [MGI Ref ID J:9438]

Sicinski P; Geng Y; Ryder-Cook AS; Barnard EA; Darlison MG; Barnard PJ. 1989. The molecular basis of muscular dystrophy in the mdx mouse: a point mutation. Science 244(4912):1578-80. [PubMed: 2662404]  [MGI Ref ID J:9866]

Additional References

Bertoni C; Lau C; Rando TA. 2003. Restoration of dystrophin expression in mdx muscle cells by chimeraplast-mediated exon skipping. Hum Mol Genet 12(10):1087-99. [PubMed: 12719373]  [MGI Ref ID J:83434]

Bulfield G; Moore EA; Kacser H. 1978. Genetic variation in activity of the enzymes of glycolysis and gluconeogenesis between inbred strains of mice. Genetics 89(3):551-61. [PubMed: 669257]  [MGI Ref ID J:27636]

Carretta D; Santarelli M; Sbriccoli A; Pinto F; Catini C; Minciacchi D. 2004. Spatial analysis reveals alterations of parvalbumin- and calbindin-positive local circuit neurons in the cerebral cortex of mutant mdx mice. Brain Res 1016(1):1-11. [PubMed: 15234246]  [MGI Ref ID J:91225]

D'Souza VN; Nguyen TM; Morris GE; Karges W; Pillers DA; Ray PN. 1995. A novel dystrophin isoform is required for normal retinal electrophysiology. Hum Mol Genet 4(5):837-42. [PubMed: 7633443]  [MGI Ref ID J:25211]

Deconinck AE; Rafael JA; Skinner JA; Brown SC; Potter AC; Metzinger L ; Watt DJ ; Dickson JG ; Tinsley JM ; Davies KE. 1997. Utrophin-dystrophin-deficient mice as a model for Duchenne muscular dystrophy. Cell 90(4):717-27. [PubMed: 9288751]  [MGI Ref ID J:42388]

Grady RM; Teng H; Nichol MC; Cunningham JC; Wilkinson RS; Sanes JR. 1997. Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin: a model for Duchenne muscular dystrophy. Cell 90(4):729-38. [PubMed: 9288752]  [MGI Ref ID J:42389]

Houzelstein D; Lyons GE; Chamberlain J; Buckingham ME. 1992. Localization of dystrophin gene transcripts during mouse embryogenesis. J Cell Biol 119(4):811-21. [PubMed: 1429837]  [MGI Ref ID J:3145]

Krag TO; Bogdanovich S; Jensen CJ; Fischer MD; Hansen-Schwartz J; Javazon EH; Flake AW; Edvinsson L; Khurana TS. 2004. Heregulin ameliorates the dystrophic phenotype in mdx mice. Proc Natl Acad Sci U S A 101(38):13856-60. [PubMed: 15365169]  [MGI Ref ID J:92602]

Kronqvist P; Kawaguchi N; Albrechtsen R; Xu X; Schroder HD; Moghadaszadeh B; Nielsen FC; Frohlich C; Engvall E; Wewer UM. 2002. ADAM12 alleviates the skeletal muscle pathology in mdx dystrophic mice. Am J Pathol 161(5):1535-40. [PubMed: 12414501]  [MGI Ref ID J:79904]

Kumar A; Khandelwal N; Malya R; Reid MB; Boriek AM. 2004. Loss of dystrophin causes aberrant mechanotransduction in skeletal muscle fibers. FASEB J 18(1):102-13. [PubMed: 14718391]  [MGI Ref ID J:87819]

Moghadaszadeh B; Albrechtsen R; Guo LT; Zaik M; Kawaguchi N; Borup RH; Kronqvist P; Schroder HD; Davies KE; Voit T; Nielsen FC; Engvall E; Wewer UM. 2003. Compensation for dystrophin-deficiency: ADAM12 overexpression in skeletal muscle results in increased alpha 7 integrin, utrophin and associated glycoproteins. Hum Mol Genet 12(19):2467-79. [PubMed: 12915458]  [MGI Ref ID J:85973]

Nakayama Y; Nara N; Kawakita Y; Takeshima Y; Arakawa M; Katoh M; Morita S; Iwatsuki K; Tanaka K; Okamoto S; Kitamura T; Seki N; Matsuda R; Matsuo M; Saito K; Hara T. 2004. Cloning of cDNA encoding a regeneration-associated muscle protease whose expression is attenuated in cell lines derived from Duchenne muscular dystrophy patients. Am J Pathol 164(5):1773-82. [PubMed: 15111323]  [MGI Ref ID J:89569]

Sadeghi A; Doyle AD; Johnson BD. 2002. Regulation of the cardiac L-type Ca2+ channel by the actin-binding proteins alpha-actinin and dystrophin. Am J Physiol Cell Physiol 282(6):C1502-11. [PubMed: 11997265]  [MGI Ref ID J:77042]

Tidball JG; Wehling-Henricks M. 2004. Expression of a NOS transgene in dystrophin-deficient muscle reduces muscle membrane damage without increasing the expression of membrane-associated cytoskeletal proteins. Mol Genet Metab 82(4):312-20. [PubMed: 15308129]  [MGI Ref ID J:94166]

Dmd^mdx related

't Hoen PA; de Meijer EJ; Boer JM; Vossen RH; Turk R; Maatman RG; Davies KE; van Ommen GJ; van Deutekom JC; den Dunnen JT. 2008. Generation and characterization of transgenic mice with the full-length human DMD gene. J Biol Chem 283(9):5899-907. [PubMed: 18083704]  [MGI Ref ID J:132320]

Abmayr S; Crawford RW; Chamberlain JS. 2004. Characterization of ARC, apoptosis repressor interacting with CARD, in normal and dystrophin-deficient skeletal muscle. Hum Mol Genet 13(2):213-21. [PubMed: 14645204]  [MGI Ref ID J:87688]

Acharyya S; Butchbach ME; Sahenk Z; Wang H; Saji M; Carathers M; Ringel MD; Skipworth RJ; Fearon KC; Hollingsworth MA; Muscarella P; Burghes AH; Rafael-Fortney JA; Guttridge DC. 2005. Dystrophin glycoprotein complex dysfunction: a regulatory link between muscular dystrophy and cancer cachexia. Cancer Cell 8(5):421-32. [PubMed: 16286249]  [MGI Ref ID J:103953]

Acharyya S; Villalta SA; Bakkar N; Bupha-Intr T; Janssen PM; Carathers M; Li ZW; Beg AA; Ghosh S; Sahenk Z; Weinstein M; Gardner KL; Rafael-Fortney JA; Karin M; Tidball JG; Baldwin AS; Guttridge DC. 2007. Interplay of IKK/NF-kappaB signaling in macrophages and myofibers promotes muscle degeneration in Duchenne muscular dystrophy. J Clin Invest 117(4):889-901. [PubMed: 17380205]  [MGI Ref ID J:121279]

Agbulut O; Noirez P; Butler-Browne G; Jockusch H. 2004. Specific isomyosin proportions in hyperexcitable and physiologically denervated mouse muscle. FEBS Lett 561(1-3):191-4. [PubMed: 15013776]  [MGI Ref ID J:117992]

Ahmad A; Brinson M; Hodges BL; Chamberlain JS; Amalfitano A. 2000. Mdx mice inducibly expressing dystrophin provide insights into the potential of gene therapy for duchenne muscular dystrophy Hum Mol Genet 9(17):2507-15. [PubMed: 11030755]  [MGI Ref ID J:65413]

Alameddine HS; Quantin B; Cartaud A; Dehaupas M; Mandel JL; Fardeau M. 1994. Expression of a recombinant dystrophin in mdx mice using adenovirus vector. Neuromuscul Disord 4(3):193-203. [PubMed: 7919968]  [MGI Ref ID J:19384]

Anderson JE. 2000. A role for nitric oxide in muscle repair: nitric oxide-mediated activation of muscle satellite cells. Mol Biol Cell 11(5):1859-74. [PubMed: 10793157]  [MGI Ref ID J:120497]

Anderson JE. 1991. Dystrophic changes in mdx muscle regenerating from denervation and devascularization. Muscle Nerve 14(3):268-79. [PubMed: 2041548]  [MGI Ref ID J:116345]

Anderson JE; Garrett K; Moor A; McIntosh L; Penner K. 1998. Dystrophy and myogenesis in mdx diaphragm muscle. Muscle Nerve 21(9):1153-65. [PubMed: 9703441]  [MGI Ref ID J:116331]

Anderson JE; Kao L; Bressler BH; Gruenstein E. 1990. Analysis of dystrophin in fast- and slow-twitch skeletal muscles from mdx and dy2J mice at different ages. Muscle Nerve 13(1):6-11. [PubMed: 2183046]  [MGI Ref ID J:116019]

Anderson JE; Lentz DL; Johnson RB. 1993. Recovery from disuse osteopenia coincident to restoration of muscle strength in mdx mice. Bone 14(4):625-34. [PubMed: 8274305]  [MGI Ref ID J:17563]

Anderson JE; Liu L; Kardami E. 1994. The effects of hyperthyroidism on muscular dystrophy in the mdx mouse: greater dystrophy in cardiac and soleus muscle. Muscle Nerve 17(1):64-73. [PubMed: 8264704]  [MGI Ref ID J:115865]

Anderson JE; Liu L; Kardami E; Murphy LJ. 1994. The pituitary-muscle axis in mdx dystrophic mice. J Neurol Sci 123(1-2):80-7. [PubMed: 8064326]  [MGI Ref ID J:18080]

Anderson JE; McIntosh LM; Moor AN; Yablonka-Reuveni Z. 1998. Levels of MyoD protein expression following injury of mdx and normal limb muscle are modified by thyroid hormone. J Histochem Cytochem 46(1):59-67. [PubMed: 9407021]  [MGI Ref ID J:45263]

Anderson JL; Head SI; Morley JW. 2003. Altered inhibitory input to Purkinje cells of dystrophin-deficient mice. Brain Res 982(2):280-3. [PubMed: 12915262]  [MGI Ref ID J:85437]

Anderson JL; Head SI; Morley JW. 2004. Long-term depression is reduced in cerebellar Purkinje cells of dystrophin-deficient mdx mice. Brain Res 1019(1-2):289-92. [PubMed: 15306266]  [MGI Ref ID J:91994]

Angoli D; Corona P; Baresi R; Mora M; Wanke E. 1997. Laminin-alpha2 but not -alpha1-mediated adhesion of human (Duchenne) and murine (mdx) dystrophic myotubes is seriously defective. FEBS Lett 408(3):341-4. [PubMed: 9188790]  [MGI Ref ID J:40762]

Asai A; Sahani N; Kaneki M; Ouchi Y; Martyn JA; Yasuhara SE. 2007. Primary role of functional ischemia, quantitative evidence for the two-hit mechanism, and phosphodiesterase-5 inhibitor therapy in mouse muscular dystrophy. PLoS ONE 2(8):e806. [PubMed: 17726536]  [MGI Ref ID J:129397]

Asselin I; Tremblay M; Vilquin JT; Guerette B; Roy R; Tremblay JP. 1995. Quantification of normal dystrophin mRNA following myoblast transplantation in mdx mice. Muscle Nerve 18(9):980-6. [PubMed: 7643878]  [MGI Ref ID J:28866]

Asselin I; Tremblay M; Vilquin JT; Guerette B; Tremblay JP. 1994. Polymerase chain reaction-based assay to assess the success of myoblast transplantation in mdx mice. Transplant Proc 26(6):3389. [PubMed: 7527970]  [MGI Ref ID J:21945]

Attal P; Lambert F; Marchand-Adam S; Bobin S; Pourny JC; Chemla D; Lecarpentier Y; Coirault C. 2000. Severe mechanical dysfunction in pharyngeal muscle from adult mdx mice. Am J Respir Crit Care Med 162(1):278-81. [PubMed: 10903254]  [MGI Ref ID J:103161]

Auda-Boucher G; Rouaud T; Lafoux A; Levitsky D; Huchet-Cadiou C; Feron M; Guevel L; Talon S; Fontaine-Perus J; Gardahaut MF. 2007. Fetal muscle-derived cells can repair dystrophic muscles in mdx mice. Exp Cell Res 313(5):997-1007. [PubMed: 17275812]  [MGI Ref ID J:119767]

Augustin M; Klopp N; Ewald K; Jockusch H. 1998. A multicopy c-Myc transgene as a nuclear label: overgrowth of Myctg50 cells in allophenic mice. Cell Biol Int 22(6):401-11. [PubMed: 10328848]  [MGI Ref ID J:127669]

Austin L; Bower JJ; Bennett TM; Lynch GS; Kapsa R; White JD; Barnard W; Gregorevic P; Byrne E. 2000. Leukemia inhibitory factor ameliorates muscle fiber degeneration in the mdx mouse. Muscle Nerve 23(11):1700-5. [PubMed: 11054748]  [MGI Ref ID J:116201]

Azzena GB; Mancinelli R. 1999. Nitric oxide regenerates the normal colonic peristaltic activity in mdx dystrophic mouse. Neurosci Lett 261(1-2):9-12. [PubMed: 10081914]  [MGI Ref ID J:107984]

Baccari MC; Romagnani P; Calamai F. 2000. Impaired nitrergic relaxations in the gastric fundus of dystrophic (mdx) mice. Neurosci Lett 282(1-2):105-8. [PubMed: 10713407]  [MGI Ref ID J:107938]

Badalamente MA; Stracher A. 2000. Delay of muscle degeneration and necrosis in mdx mice by calpain inhibition. Muscle Nerve 23(1):106-11. [PubMed: 10590413]  [MGI Ref ID J:116195]

Bakker AJ; Head SI; Williams DA; Stephenson DG. 1993. Ca2+ levels in myotubes grown from the skeletal muscle of dystrophic (mdx) and normal mice. J Physiol (Lond) 460:1-13. [PubMed: 8487190]  [MGI Ref ID J:15813]

Banks GB; Combs AC; Chamberlain JR; Chamberlain JS. 2008. Molecular and cellular adaptations to chronic myotendinous strain injury in mdx mice expressing a truncated dystrophin. Hum Mol Genet 17(24):3975-86. [PubMed: 18799475]  [MGI Ref ID J:142565]

Barton ER. 2006. Impact of sarcoglycan complex on mechanical signal transduction in murine skeletal muscle. Am J Physiol Cell Physiol 290(2):C411-9. [PubMed: 16162659]  [MGI Ref ID J:104967]

Barton ER; Morris L; Musaro A; Rosenthal N; Sweeney HL. 2002. Muscle-specific expression of insulin-like growth factor I counters muscle decline in mdx mice. J Cell Biol 157(1):137-48. [PubMed: 11927606]  [MGI Ref ID J:75770]

Barton-Davis ER; Cordier L; Shoturma DI; Leland SE; Sweeney HL. 1999. Aminoglycoside antibiotics restore dystrophin function to skeletal muscles of mdx mice [see comments] J Clin Invest 104(4):375-81. [PubMed: 10449429]  [MGI Ref ID J:56908]

Beedle AM; Nienaber PM; Campbell KP. 2007. Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex. J Biol Chem 282(23):16713-7. [PubMed: 17452335]  [MGI Ref ID J:122734]

Bertoni C; Lau C; Rando TA. 2003. Restoration of dystrophin expression in mdx muscle cells by chimeraplast-mediated exon skipping. Hum Mol Genet 12(10):1087-99. [PubMed: 12719373]  [MGI Ref ID J:83434]

Bhagwati S; Ghatpande A; Shafiq SA; Leung B. 1996. In situ hybridization analysis for expression of myogenic regulatory factors in regenerating muscle of mdx mouse. J Neuropathol Exp Neurol 55(5):509-14. [PubMed: 8627340]  [MGI Ref ID J:33889]

Bia BL; Cassidy PJ; Young ME; Rafael JA; Leighton B; Davies KE; Radda GK; Clarke K. 1999. Decreased myocardial nNOS, increased iNOS and abnormal ECGs in mouse models of Duchenne muscular dystrophy. J Mol Cell Cardiol 31(10):1857-62. [PubMed: 10525423]  [MGI Ref ID J:102663]

Bittner RE; Streubel B; Shorny S; Schaden G; Voit T; Hoger H. 1994. Coisogenic all-plus-one immunization: a model for identifying missing proteins in null-mutant conditions. Antibodies to dystrophin in mdx mouse after transplantation of muscle from normal coisogenic donor. Neuropediatrics 25(4):176-82. [PubMed: 7824089]  [MGI Ref ID J:23190]

Blaauw B; Mammucari C; Toniolo L; Agatea L; Abraham R; Sandri M; Reggiani C; Schiaffino S. 2008. Akt activation prevents the force drop induced by eccentric contractions in dystrophin-deficient skeletal muscle. Hum Mol Genet 17(23):3686-96. [PubMed: 18753145]  [MGI Ref ID J:141001]

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