JAX Mouse Strain Datasheet - 016622

STOCK Utrn^tm1Jrs Dmd^mdx/J

Stock No:: 016622 |; mdx/utrn -

Cryo Recovery

Overview

Also Known As: mdx/utrn -

Mice homozygous for the Utrn^tm1Jrs and Dmd^mdx exhibit an onset of skeletal muscle dystrophy as early as 4 weeks of age and are a model for Duchenne Type Muscular Dystrophy.

Donating Investigator

Suzanne Berry, University of Illinois

Genetic overview

Genetic Background	Generation

Dmd^mdx

Allele Type	Gene Symbol	Gene Name
Spontaneous	Dmd	dystrophin, muscular dystrophy

Allele Type	Gene Symbol	Gene Name
Targeted (Null/Knockout)	Utrn	utrophin

Research Applications

Cardiovascular Research
Neurobiology Research
Research Tools
Cell Biology Research
Sensorineural Research

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Details

Detailed Description

Mice homozygous for the Utrn^tm1Jrs and Dmd^mdx alleles have a lifespan of 4 to 20 weeks, with only 50% surviving beyond 8 weeks of age. As early as 3 weeks of age homozygotes display growth delays, are smaller than wildtype controls and exhibit skeletal muscle dystrophy with abnormal waddling gait at 4 weeks of age. At 10 weeks of age, double mutants exhibit skeletal muscle degeneration, necrosis and interstitial fibrosis, as well as abnormal echocardiography results. By 15 weeks of age, the double homozygotes develop dilated cardiomyopathy. The double mutants exhibit kyphosis, compromised systolic and diastolic function, progressive fibrosis throughout the heart, cardiomyocyte membrane damage, ventricular cellular necrosis, and disorganized mitochondria and myofibrils in cardiomyocytes.

Development

These double mutant mice were produced by breeding mice carrying the Utrn^tm1Jrs targeted mutation and the Dmd^mdx spontaneous mutation.

To generate the Utrn^tm1Jrs targeted mutation, a targeting vector containing PGK-Neo cassette was used to disrupt an exon encoding the beginning of the cysteine-rich region of the protein. The construct was electroporated into 129 derived R1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into recipient blastocysts. The mice were maintained on a mixed B6;129 background.

The Dmd^mdx spontaneous mutation 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 Dmd^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). A C-to-T transition occurred at position 3185, resulting in a termination codon in place of a glutamine codon.

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Selected References

Chun JL; O'Brien R; Berry SE. 2012. Cardiac dysfunction and pathology in the dystrophin and utrophin-deficient mouse during development of dilated cardiomyopathy. Neuromuscul Disord 22(4):368-79. PubMed: 22266080 MGI: J:187876

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Genetics

Dmd^mdx

Allele Symbol: Dmd^mdx

Allele Name	X linked muscular dystrophy
Allele Type	Spontaneous
Allele Synonym(s)	mdx; pke; pyruvate kinase expression
Gene Symbol and Name	Dmd, dystrophin, muscular dystrophy
Gene Synonym(s)	BMD; CMD3B; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272; Dp427; Dp71; Duchenne muscular dystrophy; MRX85; X-linked muscular dystrophy; X-linked muscular dystrophy; dys; mdx; mdx; pke; pke; pyruvate kinase expression
Strain of Origin	C57BL/10ScSn
Chromosome	X
Molecular Note	This mutation arose in 1981 in a C57BL/10ScSn colony at University of Leicester. A C-to-T transition occurred at position 3185, resulting in a termination codon in place of a glutamine codon. This mutation is predicted to produce a truncated protein.

Utrn^tm1Jrs

Allele Symbol: Utrn^tm1Jrs

Allele Name	targeted mutation 1, Joshua R Sanes
Allele Type	Targeted (Null/Knockout)
Allele Synonym(s)	uko; utrn-
Gene Symbol and Name	Utrn, utrophin
Gene Synonym(s)	AA589569; DMDL; DRP; DRP1; Dmdl; Dmdl; G-utrophin; dystrophin-like; expressed sequence AA589569
Strain of Origin	(129X1/SvJ x 129S1/Sv)F1-Kitl<+>
Chromosome	10
Molecular Note	A neomycin resistance cassette replaced 2.7 kb of sequence including 52 bp of the exon that encodes the beginning of the C-terminal cysteine rich region. RT-PCR analysis demonstrated that a mutant transcript carrying the deletion was made in homozygous and heterozygous mice. Western blot analysis on extracts of muscle or lung tissue derived from homozygous mice confirmed that no detectable protein was expressed from this allele.

Disease/Phenotype

Disease Terms

Research Areas By Genotype

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

Cardiovascular Research
- Heart Abnormalities
- Other
Neurobiology Research
- Muscular Dystrophy
  - Duchenne type
Research Tools
- Cardiovascular Research
- Neurobiology Research

Genotype: Dmd^mdx related

Cell Biology Research
- Signal Transduction
Neurobiology Research
- Muscular Dystrophy
  - Duchenne type
Sensorineural Research
- Cataracts

Mammalian Phenotype Terms by Genotype

The following phenotype information is associated with a similar, but not exact match to this JAX^® Mice strain

Genotype: Dmd^mdx/Dmd^mdx Utrn^tm1Jrs/Utrn^tm1Jrs
involves: 129S1/Sv * 129X1/SvJ * C57BL/10ScSn

mortality/aging

premature death
- die between 4 and 14 weeks of age, with only half surviving beyond 8 weeks
- lifespan is 4-20 weeks

growth/size/body region phenotype

decreased body size
- significantly smaller by 4 weeks of age

postnatal growth retardation
- grow more slowly after 3 weeks of age

muscle phenotype

abnormal muscle physiology

abnormal muscle relaxation
- relaxation time of muscle (time from peak force to baseline) is greatly prolonged

cardiomyopathy
- develop moderate to severe cardiomyopathy

dystrophic muscle
- begin to exhibit symptoms of skeletal muscle disease at 4 weeks of age and severity progresses with age
- develop severe skeletal muscle dystrophy

muscle degeneration
- muscle degeneration begins earlier than in single Dmd mutants

muscle weakness
- sternomastoid muscle generates only 40-60% as much tension as controls in response to stimulation of its nerve, indicating weakness

skeletal muscle interstitial fibrosis
- seen by 10 weeks of age

skeletal muscle necrosis
- necrosis begins earlier and persists longer than in single Dmd mutants and leads to fibrosis

cardiovascular system phenotype

cardiomyopathy
- develop moderate to severe cardiomyopathy

myocardial necrosis
- large areas of necrotic myocytes with infiltrating mononuclear cells are present in 5 of 9 mutant hearts examined at 8 to 11 weeks of age
- necrosis is most prominent in the epicardial surface of the right ventricle, but is seen throughout both ventricles, however ventricles are not dilated or hypertrophic

nervous system phenotype

abnormal neuromuscular synapse morphology
- distribution of acetylcholine receptors within the synapse branches is abnormal, with a patchy distribution
- exhibit fewer junctional folds in the neuromuscular junction

limbs/digits/tail phenotype

abnormal limb morphology
- exhibit contracted and stiff limbs at 4 weeks of age
- severe limb contractures

skeleton phenotype

kyphosis

behavior/neurological phenotype

abnormal gait
- abnormal waddling gait is seen at 4 weeks of age

References

Chun JL; O'Brien R; Berry SE. 2012. Cardiac dysfunction and pathology in the dystrophin and utrophin-deficient mouse during development of dilated cardiomyopathy. Neuromuscul Disord 22(4):368-79. PubMed: 22266080 MGI: J:187876

Additional - 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: 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: 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: 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: J:121279
Acuna MJ; Pessina P; Olguin H; Cabrera D; Vio CP; Bader M; Munoz-Canoves P; Santos RA; Cabello-Verrugio C; Brandan E. 2014. Restoration of muscle strength in dystrophic muscle by angiotensin-1-7 through inhibition of TGF-beta signalling. Hum Mol Genet 23(5):1237-49. PubMed: 24163134 MGI: J:206216
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: 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: J:65413
Al-Rewashdy H; Ljubicic V; Lin W; Renaud JM; Jasmin BJ. 2015. Utrophin A is essential in mediating the functional adaptations of mdx mouse muscle following chronic AMPK activation. Hum Mol Genet 24(5):1243-55. PubMed: 25324540 MGI: J:219231
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: J:19384
Alfaro LA; Dick SA; Siegel AL; Anonuevo AS; McNagny KM; Megeney LA; Cornelison DD; Rossi FM. 2011. CD34 promotes satellite cell motility and entry into proliferation to facilitate efficient skeletal muscle regeneration. Stem Cells 29(12):2030-41. PubMed: 21997891 MGI: J:190197
Altamirano F; Perez CF; Liu M; Widrick J; Barton ER; Allen PD; Adams JA; Lopez JR. 2014. Whole body periodic acceleration is an effective therapy to ameliorate muscular dystrophy in mdx mice. PLoS One 9(9):e106590. PubMed: 25181488 MGI: J:223267
Altamirano F; Valladares D; Henriquez-Olguin C; Casas M; Lopez JR; Allen PD; Jaimovich E. 2013. Nifedipine treatment reduces resting calcium concentration, oxidative and apoptotic gene expression, and improves muscle function in dystrophic mdx mice. PLoS One 8(12):e81222. PubMed: 24349043 MGI: J:209732
Amenta AR; Yilmaz A; Bogdanovich S; McKechnie BA; Abedi M; Khurana TS; Fallon JR. 2011. Biglycan recruits utrophin to the sarcolemma and counters dystrophic pathology in mdx mice. Proc Natl Acad Sci U S A 108(2):762-7. PubMed: 21187385 MGI: J:170566
Amirouche A; Tadesse H; Lunde JA; Belanger G; Cote J; Jasmin BJ. 2013. Activation of p38 signaling increases utrophin A expression in skeletal muscle via the RNA-binding protein KSRP and inhibition of AU-rich element-mediated mRNA decay: implications for novel DMD therapeutics. Hum Mol Genet 22(15):3093-111. PubMed: 23575223 MGI: J:198527
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: J:120497
Anderson JE. 1991. Dystrophic changes in mdx muscle regenerating from denervation and devascularization. Muscle Nerve 14(3):268-79. PubMed: 2041548 MGI: J:116345
Anderson JE; Bressler BH; Ovalle WK. 1988. Functional regeneration in the hindlimb skeletal muscle of the mdx mouse. J Muscle Res Cell Motil 9(6):499-515. PubMed: 3209690 MGI: J:152749
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: 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: 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: 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: 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: 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: 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: 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: 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: J:40762
Annese T; Corsi P; Ruggieri S; Tamma R; Marinaccio C; Picocci S; Errede M; Specchia G; De Luca A; Frassanito MA; Desantis V; Vacca A; Ribatti D; Nico B. 2016. Isolation and characterization of neural stem cells from dystrophic mdx mouse. Exp Cell Res :. PubMed: 27015747 MGI: J:230853
Apolinario LM; De Carvalho SC; Santo Neto H; Marques MJ. 2015. Long-Term Therapy With Omega-3 Ameliorates Myonecrosis and Benefits Skeletal Muscle Regeneration in Mdx Mice. Anat Rec (Hoboken) 298(9):1589-96. PubMed: 26011009 MGI: J:224775
Ardite E; Perdiguero E; Vidal B; Gutarra S; Serrano AL; Munoz-Canoves P. 2012. PAI-1-regulated miR-21 defines a novel age-associated fibrogenic pathway in muscular dystrophy. J Cell Biol 196(1):163-75. PubMed: 22213800 MGI: J:179966
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: J:129397
Ascah A; Khairhallah M; Daussin F; Bourcier-Lucas C; Godin R; Allen BG; Petrof BJ; Des Rosiers C; Burelle Y. 2010. STRESS-INDUCED OPENING OF THE PERMEABILITY TRANSITION PORE IN THE DYSTROPHIN-DEFICIENT HEART IS ATTENUATED BY ACUTE TREATMENT WITH SILDENAFIL. Am J Physiol Heart Circ Physiol :. PubMed: 20971771 MGI: J:166227
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: 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: 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: 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: 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: 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: 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: 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: 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: J:116195
Bagher P; Duan D; Segal SS. 2011. Evidence for impaired neurovascular transmission in a murine model of Duchenne muscular dystrophy. J Appl Physiol 110(3):601-9. PubMed: 21109597 MGI: J:185890
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 460:1-13. PubMed: 8487190 MGI: J:15813
Ballarino M; Cazzella V; D'Andrea D; Grassi L; Bisceglie L; Cipriano A; Santini T; Pinnaro C; Morlando M; Tramontano A; Bozzoni I. 2015. Novel long noncoding RNAs (lncRNAs) in myogenesis: a miR-31 overlapping lncRNA transcript controls myoblast differentiation. Mol Cell Biol 35(4):728-36. PubMed: 25512605 MGI: J:224300
Ballmann C; Denney TS; Beyers RJ; Quindry T; Romero M; Amin R; Selsby JT; Quindry JC. 2017. Lifelong quercetin enrichment and cardioprotection in Mdx/Utrn+/- mice. Am J Physiol Heart Circ Physiol 312(1):H128-H140. PubMed: 27836895 MGI: J:239409
Baltgalvis KA; Jaeger MA; Fitzsimons DP; Thayer SA; Lowe DA; Ervasti JM. 2011. Transgenic overexpression of gamma-cytoplasmic actin protects against eccentric contraction-induced force loss in mdx mice. Skelet Muscle 1(1):32. PubMed: 21995957 MGI: J:183731
Banks GB; Chamberlain JS; Froehner SC. 2009. Truncated dystrophins can influence neuromuscular synapse structure. Mol Cell Neurosci 40(4):433-41. PubMed: 19171194 MGI: J:146866
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: J:142565
Barnabei MS; Metzger JM. 2012. Ex vivo stretch reveals altered mechanical properties of isolated dystrophin-deficient hearts. PLoS One 7(3):e32880. PubMed: 22427904 MGI: J:186918
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: 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: 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: J:56908
Bates G; Sigurdardottir S; Kachmar L; Zitouni NB; Benedetti A; Petrof BJ; Rassier D; Lauzon AM. 2013. Molecular, cellular, and muscle strip mechanics of the mdx mouse diaphragm. Am J Physiol Cell Physiol 304(9):C873-80. PubMed: 23426972 MGI: J:196389
Beastrom N; Lu H; Macke A; Canan BD; Johnson EK; Penton CM; Kaspar BK; Rodino-Klapac LR; Zhou L; Janssen PM; Montanaro F. 2011. mdx(5cv) Mice Manifest More Severe Muscle Dysfunction and Diaphragm Force Deficits than Do mdx Mice. Am J Pathol 179(5):2464-74. PubMed: 21893021 MGI: J:177391
Bechir N; Pecchi E; Vilmen C; Le Fur Y; Amthor H; Bernard M; Bendahan D; Giannesini B. 2016. ActRIIB blockade increases force-generating capacity and preserves energy supply in exercising mdx mouse muscle in vivo. FASEB J 30(10):3551-3562. PubMed: 27416839 MGI: J:239706
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: J:122734
Belanto JJ; Mader TL; Eckhoff MD; Strandjord DM; Banks GB; Gardner MK; Lowe DA; Ervasti JM. 2014. Microtubule binding distinguishes dystrophin from utrophin. Proc Natl Acad Sci U S A 111(15):5723-8. PubMed: 24706788 MGI: J:208636
Belanto JJ; Olthoff JT; Mader TL; Chamberlain CM; Nelson DM; McCourt PM; Talsness DM; Gundersen GG; Lowe DA; Ervasti JM. 2016. Independent variability of microtubule perturbations associated with dystrophinopathy. Hum Mol Genet 25(22):4951-4961. PubMed: 28171583 MGI: J:241782
Bellinger AM; Reiken S; Carlson C; Mongillo M; Liu X; Rothman L; Matecki S; Lacampagne A; Marks AR. 2009. Hypernitrosylated ryanodine receptor calcium release channels are leaky in dystrophic muscle. Nat Med 15(3):325-30. PubMed: 19198614 MGI: J:146474
Bentzinger CF; Romanino K; Cloetta D; Lin S; Mascarenhas JB; Oliveri F; Xia J; Casanova E; Costa CF; Brink M; Zorzato F; Hall MN; Ruegg MA. 2008. Skeletal muscle-specific ablation of raptor, but not of rictor, causes metabolic changes and results in muscle dystrophy. Cell Metab 8(5):411-24. PubMed: 19046572 MGI: J:143748
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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: 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: J:23190
Blaauw B; Agatea L; Toniolo L; Canato M; Quarta M; Dyar KA; Danieli-Betto D; Betto R; Schiaffino S; Reggiani C. 2010. Eccentric contractions lead to myofibrillar dysfunction in muscular dystrophy. J Appl Physiol 108(1):105-11. PubMed: 19910334 MGI: J:185865
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: J:141001
Blain A; Greally E; Laval S; Blamire A; Straub V; MacGowan GA. 2013. Beta-blockers, left and right ventricular function, and in-vivo calcium influx in muscular dystrophy cardiomyopathy. PLoS One 8(2):e57260. PubMed: 23437355 MGI: J:199333
Blanchet E; Annicotte JS; Pradelli LA; Hugon G; Matecki S; Mornet D; Rivier F; Fajas L. 2012. E2F transcription factor-1 deficiency reduces pathophysiology in the mouse model of Duchenne muscular dystrophy through increased muscle oxidative metabolism. Hum Mol Genet 21(17):3910-7. PubMed: 22678059 MGI: J:185978
Bloom TJ. 2005. Age-related alterations in cyclic nucleotide phosphodiesterase activity in dystrophic mouse leg muscle. Can J Physiol Pharmacol 83(11):1055-60. PubMed: 16391714 MGI: J:110508
Bloom TJ. 2002. Cyclic nucleotide phosphodiesterase isozymes expressed in mouse skeletal muscle. Can J Physiol Pharmacol 80(12):1132-5. PubMed: 12564638 MGI: J:102408
Bobet J; Mooney RF; Gordon T. 1998. Force and stiffness of old dystrophic (mdx) mouse skeletal muscles. Muscle Nerve 21(4):536-9. PubMed: 9533791 MGI: J:116778
Boettger T; Wust S; Nolte H; Braun T. 2014. The miR-206/133b cluster is dispensable for development, survival and regeneration of skeletal muscle. Skelet Muscle 4(1):23. PubMed: 25530839 MGI: J:241493
Boland B; Himpens B; Casteels R; Gillis JM. 1993. Lack of dystrophin but normal calcium homeostasis in smooth muscle from dystrophic mdx mice. J Muscle Res Cell Motil 14(1):133-9. PubMed: 8478423 MGI: J:13345
Boland B; Himpens B; Denef JF; Gillis JM. 1995. Site-dependent pathological differences in smooth muscles and skeletal muscles of the adult mdx mouse. Muscle Nerve 18(6):649-57. PubMed: 7753128 MGI: J:26592
Boonsanay V; Zhang T; Georgieva A; Kostin S; Qi H; Yuan X; Zhou Y; Braun T. 2016. Regulation of Skeletal Muscle Stem Cell Quiescence by Suv4-20h1-Dependent Facultative Heterochromatin Formation. Cell Stem Cell 18(2):229-42. PubMed: 26669898 MGI: J:239999
Boppart MD; Burkin DJ; Kaufman SJ. 2011. Activation of AKT signaling promotes cell growth and survival in alpha7beta1 integrin-mediated alleviation of muscular dystrophy. Biochim Biophys Acta 1812(4):439-46. PubMed: 21216283 MGI: J:170661
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Zhao J; Yoshioka K; Miike T; Miyatake M. 1993. Developmental studies of dystrophin-positive fibers in mdx, and DRP localization. J Neurol Sci 114(1):104-8. PubMed: 8433092 MGI: J:3667
Zhao X; Moloughney JG; Zhang S; Komazaki S; Weisleder N. 2012. Orai1 mediates exacerbated Ca(2+) entry in dystrophic skeletal muscle. PLoS One 7(11):e49862. PubMed: 23185465 MGI: J:195456
Zhou L; Porter JD; Cheng G; Gong B; Hatala DA; Merriam AP; Zhou X; Rafael JA; Kaminski HJ. 2006. Temporal and spatial mRNA expression patterns of TGF-beta1, 2, 3 and TbetaRI, II, III in skeletal muscles of mdx mice. Neuromuscul Disord 16(1):32-8. PubMed: 16373085 MGI: J:112758
Zhou L; Rafael-Fortney JA; Huang P; Zhao XS; Cheng G; Zhou X; Kaminski HJ; Liu L; Ransohoff RM. 2008. Haploinsufficiency of utrophin gene worsens skeletal muscle inflammation and fibrosis in mdx mice. J Neurol Sci 264(1-2):106-11. PubMed: 17889902 MGI: J:140282
Zhou L; Sun K; Zhao Y; Zhang S; Wang X; Li Y; Lu L; Chen X; Chen F; Bao X; Zhu X; Wang L; Tang LY; Esteban MA; Wang CC; Jauch R; Sun H; Wang H. 2015. Linc-YY1 promotes myogenic differentiation and muscle regeneration through an interaction with the transcription factor YY1. Nat Commun 6:10026. PubMed: 26658965 MGI: J:228292
Zhuang W; Eby JC; Cheong M; Mohapatra PK; Bredt DS; Disatnik MH; Rando TA. 2001. The susceptibility of muscle cells to oxidative stress is independent of nitric oxide synthase expression. Muscle Nerve 24(4):502-11. PubMed: 11268022 MGI: J:116198
van Deutekom JC; Cao B; Pruchnic R; Wickham TJ; Kovesdi I; Huard J. 1999. Extended tropism of an adenoviral vector does not circumvent the maturation-dependent transducibility of mouse skeletal muscle. J Gene Med 1(6):393-9. PubMed: 10753064 MGI: J:62398
van Putten M; Hulsker M; Nadarajah VD; van Heiningen SH; van Huizen E; van Iterson M; Admiraal P; Messemaker T; den Dunnen JT; 't Hoen PA; Aartsma-Rus A. 2012. The effects of low levels of dystrophin on mouse muscle function and pathology. PLoS One 7(2):e31937. PubMed: 22359642 MGI: J:185221
van Putten M; Hulsker M; Young C; Nadarajah VD; Heemskerk H; van der Weerd L; 't Hoen PA; van Ommen GJ; Aartsma-Rus AM. 2013. Low dystrophin levels increase survival and improve muscle pathology and function in dystrophin/utrophin double-knockout mice. FASEB J 27(6):2484-95. PubMed: 23460734 MGI: J:199957
von Maltzahn J; Renaud JM; Parise G; Rudnicki MA. 2012. Wnt7a treatment ameliorates muscular dystrophy. Proc Natl Acad Sci U S A 109(50):20614-9. PubMed: 23185011 MGI: J:193128

Additional - Utrn^tm1Jrs related

Boppart MD; Burkin DJ; Kaufman SJ. 2011. Activation of AKT signaling promotes cell growth and survival in alpha7beta1 integrin-mediated alleviation of muscular dystrophy. Biochim Biophys Acta 1812(4):439-46. PubMed: 21216283 MGI: J:170661
Burkin DJ; Wallace GQ; Milner DJ; Chaney EJ; Mulligan JA; Kaufman SJ. 2005. Transgenic expression of {alpha}7{beta}1 integrin maintains muscle integrity, increases regenerative capacity, promotes hypertrophy, and reduces cardiomyopathy in dystrophic mice. Am J Pathol 166(1):253-63. PubMed: 15632017 MGI: J:96066
Burkin DJ; Wallace GQ; Nicol KJ; Kaufman DJ; Kaufman SJ. 2001. Enhanced Expression of the alpha7beta1 Integrin Reduces Muscular Dystrophy and Restores Viability in Dystrophic Mice. J Cell Biol 152(6):1207-18. PubMed: 11257121 MGI: J:68194
Capote J; DiFranco M; Vergara JL. 2010. Excitation-contraction coupling alterations in mdx and utrophin/dystrophin double knockout mice: a comparative study. Am J Physiol Cell Physiol 298(5):C1077-86. PubMed: 20130206 MGI: J:159256
Chadwick JA; Bhattacharya S; Lowe J; Weisleder N; Rafael-Fortney JA. 2017. Renin-angiotensin-aldosterone system inhibitors improve membrane stability and change gene-expression profiles in dystrophic skeletal muscles. Am J Physiol Cell Physiol 312(2):C155-C168. PubMed: 27881412 MGI: J:239401
Connolly AM; Keeling RM; Mehta S; Pestronk A; Sanes JR. 2001. Three mouse models of muscular dystrophy: the natural history of strength and fatigue in dystrophin-, dystrophin/utrophin-, and laminin alpha2-deficient mice. Neuromuscul Disord 11(8):703-12. PubMed: 11595512 MGI: J:103061
Crosbie RH; Lebakken CS; Holt KH; Venzke DP; Straub V; Lee JC; Grady RM; Chamberlain JS; Sanes JR; Campbell KP. 1999. Membrane targeting and stabilization of sarcospan is mediated by the sarcoglycan subcomplex. J Cell Biol 145(1):153-65. PubMed: 10189375 MGI: J:77615
Deconinck N; Rafael JA; Beckers-Bleukx G; Kahn D; Deconinck AE; Davies KE; Gillis JM. 1998. Consequences of the combined deficiency in dystrophin and utrophin on the mechanical properties and myosin composition of some limb and respiratory muscles of the mouse. Neuromuscul Disord 8(6):362-70. PubMed: 9713852 MGI: J:110988
Gaedigk R; Law DJ; Fitzgerald-Gustafson KM; McNulty SG; Nsumu NN; Modrcin AC; Rinaldi RJ; Pinson D; Fowler SC; Bilgen M; Burns J; Hauschka SD; White RA. 2006. Improvement in survival and muscle function in an mdx/utrn(-/-) double mutant mouse using a human retinal dystrophin transgene. Neuromuscul Disord 16(3):192-203. PubMed: 16487708 MGI: J:112787
Gehrig SM; van der Poel C; Sayer TA; Schertzer JD; Henstridge DC; Church JE; Lamon S; Russell AP; Davies KE; Febbraio MA; Lynch GS. 2012. Hsp72 preserves muscle function and slows progression of severe muscular dystrophy. Nature 484(7394):394-8. PubMed: 22495301 MGI: J:183833
Grady RM; Grange RW; Lau KS; Maimone MM; Nichol MC; Stull JT; Sanes JR. 1999. Role for alpha-dystrobrevin in the pathogenesis of dystrophin-dependent muscular dystrophies. Nat Cell Biol 1(4):215-20. PubMed: 10559919 MGI: J:59675
Grady RM; Merlie JP; Sanes JR. 1997. Subtle neuromuscular defects in utrophin-deficient mice. J Cell Biol 136(4):871-82. PubMed: 9049252 MGI: J:39218
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: J:42389
Grady RM; Zhou H; Cunningham JM; Henry MD; Campbell KP; Sanes JR. 2000. Maturation and maintenance of the neuromuscular synapse: genetic evidence for roles of the dystrophin--glycoprotein complex. Neuron 25(2):279-93. PubMed: 10719885 MGI: J:60776
Gregorevic P; Allen JM; Minami E; Blankinship MJ; Haraguchi M; Meuse L; Finn E; Adams ME; Froehner SC; Murry CE; Chamberlain JS. 2006. rAAV6-microdystrophin preserves muscle function and extends lifespan in severely dystrophic mice. Nat Med 12(7):787-9. PubMed: 16819550 MGI: J:111980
Gutpell KM; Hrinivich WT; Hoffman LM. 2015. Skeletal muscle fibrosis in the mdx/utrn+/- mouse validates its suitability as a murine model of Duchenne muscular dystrophy. PLoS One 10(1):e0117306. PubMed: 25607927 MGI: J:225657
Haenggi T; Schaub MC; Fritschy JM. 2005. Molecular heterogeneity of the dystrophin-associated protein complex in the mouse kidney nephron: differential alterations in the absence of utrophin and dystrophin. Cell Tissue Res 319(2):299-313. PubMed: 15565469 MGI: J:105083
Haenggi T; Soontornmalai A; Schaub MC; Fritschy JM. 2004. The role of utrophin and Dp71 for assembly of different dystrophin-associated protein complexes (DPCs) in the choroid plexus and microvasculature of the brain. Neuroscience 129(2):403-13. PubMed: 15501597 MGI: J:94669
Hanft LM; Bogan DJ; Mayer U; Kaufman SJ; Kornegay JN; Ervasti JM. 2007. Cytoplasmic gamma-actin expression in diverse animal models of muscular dystrophy. Neuromuscul Disord 17(7):569-74. PubMed: 17475492 MGI: J:124551
Judge LM; Arnett AL; Banks GB; Chamberlain JS. 2011. Expression of the dystrophin isoform Dp116 preserves functional muscle mass and extends lifespan without preventing dystrophy in severely dystrophic mice. Hum Mol Genet 20(24):4978-90. PubMed: 21949353 MGI: J:177877
Knuesel I; Riban V; Zuellig RA; Schaub MC; Grady RM; Sanes JR; Fritschy JM. 2002. Increased vulnerability to kainate-induced seizures in utrophin-knockout mice. Eur J Neurosci 15(9):1474-84. PubMed: 12028357 MGI: J:89397
Koenig X; Dysek S; Kimbacher S; Mike AK; Cervenka R; Lukacs P; Nagl K; Dang XB; Todt H; Bittner RE; Hilber K. 2011. Voltage-gated ion channel dysfunction precedes cardiomyopathy development in the dystrophic heart. PLoS One 6(5):e20300. PubMed: 21677768 MGI: J:196253
Koenig X; Rubi L; Obermair GJ; Cervenka R; Dang XB; Lukacs P; Kummer S; Bittner RE; Kubista H; Todt H; Hilber K. 2014. Enhanced currents through L-type calcium channels in cardiomyocytes disturb the electrophysiology of the dystrophic heart. Am J Physiol Heart Circ Physiol 306(4):H564-73. PubMed: 24337461 MGI: J:208702
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: J:92602
Lai Y; Zhao J; Yue Y; Duan D. 2013. alpha2 and alpha3 helices of dystrophin R16 and R17 frame a microdomain in the alpha1 helix of dystrophin R17 for neuronal NOS binding. Proc Natl Acad Sci U S A 110(2):525-30. PubMed: 23185009 MGI: J:192576
Li D; Yue Y; Duan D. 2010. Marginal level dystrophin expression improves clinical outcome in a strain of dystrophin/utrophin double knockout mice. PLoS One 5(12):e15286. PubMed: 21187970 MGI: J:168677
Liu J; Milner DJ; Boppart MD; Ross RS; Kaufman SJ. 2012. beta1D chain increases alpha7beta1 integrin and laminin and protects against sarcolemmal damage in mdx mice. Hum Mol Genet 21(7):1592-603. PubMed: 22180459 MGI: J:181562
Marshall JL; Oh J; Chou E; Lee JA; Holmberg J; Burkin DJ; Crosbie-Watson RH. 2015. Sarcospan integration into laminin-binding adhesion complexes that ameliorate muscular dystrophy requires utrophin and alpha7 integrin. Hum Mol Genet 24(7):2011-22. PubMed: 25504048 MGI: J:219598
McDonald AA; Hebert SL; Kunz MD; Ralles SJ; McLoon LK. 2015. Disease course in mdx:utrophin+/- mice: comparison of three mouse models of Duchenne muscular dystrophy. Physiol Rep 3(4):. PubMed: 25921779 MGI: J:229245
McDonald AA; Kunz MD; McLoon LK. 2014. Dystrophic changes in extraocular muscles after gamma irradiation in mdx:utrophin(+/-) mice. PLoS One 9(1):e86424. PubMed: 24466085 MGI: J:212562
McNally EM; Kaltman JR; Benson DW; Canter CE; Cripe LH; Duan D; Finder JD; Groh WJ; Hoffman EP; Judge DP; Kertesz N; Kinnett K; Kirsch R; Metzger JM; Pearson GD; Rafael-Fortney JA; Raman SV; Spurney CF; Targum SL; Wagner KR; Markham LW. 2015. Contemporary cardiac issues in Duchenne muscular dystrophy. Working Group of the National Heart, Lung, and Blood Institute in collaboration with Parent Project Muscular Dystrophy. Circulation 131(18):1590-8. PubMed: 25940966 MGI: J:234452
Patton BL; Cunningham JM; Thyboll J; Kortesmaa J; Westerblad H; Edstrom L; Tryggvason K; Sanes JR. 2001. Properly formed but improperly localized synaptic specializations in the absence of laminin alpha4. Nat Neurosci 4(6):597-604. PubMed: 11369940 MGI: J:77657
Swiderski K; Shaffer SA; Gallis B; Odom GL; Arnett AL; Scott Edgar J; Baum DM; Chee A; Naim T; Gregorevic P; Murphy KT; Moody J; Goodlett DR; Lynch GS; Chamberlain JS. 2014. Phosphorylation within the cysteine-rich region of dystrophin enhances its association with beta-dystroglycan and identifies a potential novel therapeutic target for skeletal muscle wasting. Hum Mol Genet 23(25):6697-711. PubMed: 25082828 MGI: J:216212
Verma M; Asakura Y; Hirai H; Watanabe S; Tastad C; Fong GH; Ema M; Call JA; Lowe DA; Asakura A. 2010. Flt-1 haploinsufficiency ameliorates muscular dystrophy phenotype by developmentally increased vasculature in mdx mice. Hum Mol Genet 19(21):4145-59. PubMed: 20705734 MGI: J:164891
Wehling-Henricks M; Tidball JG. 2011. Neuronal nitric oxide synthase-rescue of dystrophin/utrophin double knockout mice does not require nNOS localization to the cell membrane. PLoS One 6(10):e25071. PubMed: 22003386 MGI: J:179610
Welser JV; Rooney JE; Cohen NC; Gurpur PB; Singer CA; Evans RA; Haines BA; Burkin DJ. 2009. Myotendinous junction defects and reduced force transmission in mice that lack alpha7 integrin and utrophin. Am J Pathol 175(4):1545-54. PubMed: 19729483 MGI: J:153056
Zhou L; Rafael-Fortney JA; Huang P; Zhao XS; Cheng G; Zhou X; Kaminski HJ; Liu L; Ransohoff RM. 2008. Haploinsufficiency of utrophin gene worsens skeletal muscle inflammation and fibrosis in mdx mice. J Neurol Sci 264(1-2):106-11. PubMed: 17889902 MGI: J:140282

Also Known As: mdx/utrn -

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Dmdmdx

Allele Symbol: Dmdmdx

Utrntm1Jrs

Allele Symbol: Utrntm1Jrs

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

Genotype: Dmdmdx related

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Genotype: Dmdmdx/Dmdmdx Utrntm1Jrs/Utrntm1Jrsinvolves: 129S1/Sv * 129X1/SvJ * C57BL/10ScSn

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Dmd^mdx

Allele Symbol: Dmd^mdx

Utrn^tm1Jrs

Allele Symbol: Utrn^tm1Jrs

Genotype: Dmd^mdx related

Genotype: Dmd^mdx/Dmd^mdx Utrn^tm1Jrs/Utrn^tm1Jrs
involves: 129S1/Sv * 129X1/SvJ * C57BL/10ScSn

Additional - Dmd^mdx related

Additional - Utrn^tm1Jrs related