Strain Name:

MYD/Le-Largemyd/J

Stock Number:

018305

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Availability:

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These Largemyd mice are a model of congenital muscular dystrophy type 1D (MDC1D; also called human α-dystroglycan glycosylation-deficient muscular dystrophy).

Description

Strain Information

Type Mutant Strain; Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Type Inbred Strain;
Additional information on Inbred Strains.
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Mating SystemWild-type x Heterozygote         (Female x Male)   26-MAR-13
Mating SystemHeterozygote x Wild-type         (Female x Male)   26-MAR-13
Specieslaboratory mouse

Description
The spontaneous autosomal recessive mutation myodystrophy (myd) is a deletion in exons 5-7 of the glycotransferase gene (Large) on chromosome 8; causing a frameshift and premature stop codon before the first two catalytic domains. This Largemyd mutation results in abnormal glycosylation of its substrate α-dystroglycan.
Largemyd mice are a model of Congenital muscular dystrophy type 1D (MDC1D; also called human α-dystroglycan glycosylation-deficient muscular dystrophy). MYD/Le-Largemyd homozygotes exhibit a progressive myopathy, abnormal posture, thoracic kyphosis, calcium deposits in muscle, loss of Schwann cells and myelin, central nervous system defects, and reduced growth. Original characterization of these mice reported that myodystrophic (homozygous) mice were fertile but reproduction was very poor, and the mean lifespan of homozygotes that survived weaning was 17 weeks with a range of 5 to 39 weeks (Lane PW, Beamer TC, and Myers DD. J Hered. 1976. 67:135). When breeding MYD/Le-Largemyd heterozygotes together at The Jackson Laboratory Repository, MYD/Le-Largemyd homozygous animals were small and died early.
Of note, Largemyd homozygotes on different genetic backgrounds are also described with sensorineural hearing loss (B6C3Fe) and eye defects (C57BL/6). Whether MYD/Le-Largemyd homozygotes manifest these defects is not specifically determined.

Development
The spontaneous autosomal recessive mutation myodystrophy (myd) is a deletion in exons 5-7 of the glycotransferase gene (Large) on chromosome 8; causing a frameshift and premature stop codon before the first two catalytic domains. The history of Stock No. 018305 is described below.

The myodystrophy mutation (Largemyd) was discovered at The Jackson Laboratory in 1963 on the lethal spotting stock LS/LeJ (Stock No. 000262; which had been imported from University College, London in 1961). The first myodystrophic male was outcrossed to a C57BL/6J female. Matings of a homozygous mouse with a heterozygous mouse were carried out as often as possible (or as heterozygous pairs). The Largemyd mutation was found on chromosome 8 in close linkage with the radiation-induced oligosyndactylism mutation (Os; heterozygotes are fertile with fused toes and homozygotes die in utero). As such, a Largemyd/+ male (generation F38) was bred to an ROP inbred female harboring the Os and pintail mutations (Stock No. 000267); this allowed for maintenance/tracking of the Largemyd mutation by Os phenotype). After three sibling matings, oligosyndactylic animals (heterozygous for the Os mutation; Os/+) were crossed seven times to the +/+ members of the strain. At N7, an Os/+ mouse was again crossed to a Largemyd/+ mouse to establish the Os and Largemyd mutations in repulsion. This repulsion strain (Stock No. 000300) was subsequently maintained by breeding oligosyndactylic siblings (Os +/+ Largemyd) together. Stock No. 000300 was cryopreserved in 1981 by mating Os +/+ Largemyd males at N7F22-F27 to non Os (+ +/+ ?) females.

In 2012, heterozygous Largemyd mice without fused toes (wildtype at the Os locus) were obtained and bred together as Stock No. 018305.

Control Information

  Control
   Wild-type from the colony
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Largemyd allele
000226   B6C3Fe a/a-Largemyd/J
000300   MYD/Le-Os +/+ Largemyd/J
View Strains carrying   Largemyd     (2 strains)

Strains carrying other alleles of Large
005350   B6.CAST(Cg)-Largevls/Pjn
002491   B6.Cg-Largeenr-Tg(MpbReg)36Pop/J
008581   STOCK Largemyd-3J/GrsrJ
View Strains carrying other alleles of Large     (3 strains)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
Muscular Dystrophy-Dystroglycanopathy (congenital with Brain and Eye Anomalies), Type A, 6; MDDGA6
- Model with phenotypic similarity to human disease where etiologies are distinct. Human genes are associated with this disease. Orthologs of these genes do not appear in the mouse genotype(s).
Facioscapulohumeral Muscular Dystrophy 1; FSHD1
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Muscular Dystrophy-Dystroglycanopathy (congenital with Brain and Eye Anomalies), Type A, 1; MDDGA1   (LARGE)
Muscular Dystrophy-Dystroglycanopathy (congenital with Mental Retardation), Type B, 6; MDDGB6   (LARGE)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

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

Largemyd/Large+

        B6C3Fe a/a-Largemyd/J
  • behavior/neurological phenotype
  • limb grasping
    • some aging animals exhibit atypical hindlimb posture and movements when lifted by the tail   (MGI Ref ID J:27793)

Largemyd/Largemyd

        MYD/Le-Os +/+ Largemyd/J
  • mortality/aging
  • premature death
    • average life span 17.25 weeks, range 5-39 weeks   (MGI Ref ID J:5670)
  • behavior/neurological phenotype
  • abnormal gait
    • hind limbs held close to the body producing a short, shuffling gait   (MGI Ref ID J:5670)
    • hind limbs never extended and dragged   (MGI Ref ID J:5670)
  • limb grasping
    • involves hind limbs   (MGI Ref ID J:5670)
    • adduction of hind legs, flexion of the knee, ankles and toes   (MGI Ref ID J:5670)
    • severe contraction of hind limbs sometimes by 3-4 months of age   (MGI Ref ID J:5670)
  • muscle phenotype
  • abnormal muscle morphology   (MGI Ref ID J:5670)
    • abnormal skeletal muscle fiber morphology
      • variable fiber size   (MGI Ref ID J:5670)
      • loss of striation   (MGI Ref ID J:5670)
      • central migration of nuclei   (MGI Ref ID J:5670)
      • nuclear "rowing"   (MGI Ref ID J:5670)
    • calcified muscle
      • elevated calcium levels in skeletal muscles, particularly the diaphragm   (MGI Ref ID J:12034)
      • heart not affected   (MGI Ref ID J:12034)
    • dystrophic muscle   (MGI Ref ID J:5670)
    • muscle degeneration   (MGI Ref ID J:5670)
  • abnormal muscle physiology   (MGI Ref ID J:5670)
    • myopathy
      • diffuse and progressive myopathy   (MGI Ref ID J:5670)
      • widely distributed focal lesions in skeletal muscles as early as 16 days of age   (MGI Ref ID J:5670)
    • myositis
      • mononuclear cell infiltration of areas surrounding degenerating fibers   (MGI Ref ID J:5670)
  • nervous system phenotype
  • abnormal myelination
    • areas where nerves are completely deficient in myelin   (MGI Ref ID J:5974)
  • abnormal spinal nerve morphology
    • areas completely deficient in myelin   (MGI Ref ID J:5974)
    • not every root is affected   (MGI Ref ID J:5974)
    • observed in dorsal roots T13 to S1 and Ventral roots L1 to S1 (except L5)   (MGI Ref ID J:5974)
    • L3 and L4 ventral roots most severely affected   (MGI Ref ID J:5974)
  • absent Schwann cells
    • usually unmyelinated nerves lack Schwann cells but sometimes Schwann cells present but lacking myelin   (MGI Ref ID J:5974)
  • growth/size/body phenotype
  • decreased body weight
    • organ weights reduced comparably with reduced body weigh   (MGI Ref ID J:5670)
  • postnatal growth retardation
    • very severe at weaning   (MGI Ref ID J:5670)
    • growth improved after weaning but mice always small   (MGI Ref ID J:5670)
  • skeleton phenotype
  • abnormal bone structure
    • thinning of all bones examined   (MGI Ref ID J:12034)
  • kyphosis
    • thoracic kyphosis by 6-8 weeks of age   (MGI Ref ID J:5670)
    • becoming progressively worse with age   (MGI Ref ID J:5670)
  • reproductive system phenotype
  • reduced fertility
    • although not sterile, reproduction is very poor   (MGI Ref ID J:5670)
  • immune system phenotype
  • myositis
    • mononuclear cell infiltration of areas surrounding degenerating fibers   (MGI Ref ID J:5670)
  • digestive/alimentary phenotype
  • abnormal tongue morphology
    • musculature of tongue not affected until later   (MGI Ref ID J:5670)
    • subepithelial fibrosis in tongues of older mice   (MGI Ref ID J:5670)
  • craniofacial phenotype
  • abnormal tongue morphology
    • musculature of tongue not affected until later   (MGI Ref ID J:5670)
    • subepithelial fibrosis in tongues of older mice   (MGI Ref ID J:5670)

Largemyd/Largemyd

        B6.Cg-Largemyd/Pjn
  • vision/eye phenotype
  • abnormal eye electrophysiology
    • amplitude of b-wave responses is reduced and delayed at all flash intensities in 2 month old mice   (MGI Ref ID J:100214)
    • larger negative polarity a-wave in response to intermediate flash intensities in 2 month old mice   (MGI Ref ID J:100214)
    • maximum amplitude of a-wave reduced in response to highest flash intensities in 2 month old mice   (MGI Ref ID J:100214)
  • abnormal retinal layer morphology   (MGI Ref ID J:100214)
    • disorganized retinal outer plexiform layer
      • layer is disorganized with a reduction in synaptic complexes   (MGI Ref ID J:100214)
      • mitochondria are swollen with severe disruption of cristae   (MGI Ref ID J:100214)
      • exhibits extracelluelar edema   (MGI Ref ID J:100214)
      • layer is thinner than in control littermates   (MGI Ref ID J:100214)
    • thin retinal outer nuclear layer
      • almost 50% thinner than in controls   (MGI Ref ID J:100214)
  • muscle phenotype
  • abnormal diaphragm morphology
    • exhibits prominent interstial fibrosis with extensive degeneration and regeneration of myofibers at 1.5 months of age   (MGI Ref ID J:100214)
    • by 4 months diaphragm exhibits necrosis and fatty infiltration   (MGI Ref ID J:100214)
  • abnormal soleus morphology
    • occasional signs of fiber-type grouping in 6 month old mice   (MGI Ref ID J:100214)
  • dilated cardiomyopathy
  • cardiovascular system phenotype
  • abnormal myocardium layer morphology
    • myocardium exhibits mild to moderate areas of cardiomyocyte degeneration with mycytolysis, necrosis and interstitial fibrosis in 5 month old mice   (MGI Ref ID J:100214)
    • lesions observed in the left and right atria and ventricles   (MGI Ref ID J:100214)
  • dilated cardiomyopathy

Largemyd/Largemyd

        B6C3Fe a/a-Largemyd/J
  • muscle phenotype
  • abnormal myocardial fiber morphology
    • mutants exhibit focal patches of cardiac myocyte membrane damage   (MGI Ref ID J:169951)
  • abnormal skeletal muscle fiber morphology   (MGI Ref ID J:27793)
    • centrally nucleated skeletal muscle fibers   (MGI Ref ID J:27793)
    • increased variability of skeletal muscle fiber size
      • both fast and slow muscle fiber types show increased size variation   (MGI Ref ID J:27793)
    • skeletal muscle fiber degeneration
      • many degenerating and regenerating fibers   (MGI Ref ID J:27793)
      • foci of degeneration are typically large, irregularly shaped and involve 20-50 necrotic fibers   (MGI Ref ID J:27793)
    • skeletal muscle fiber necrosis
      • in addition to foci of 20-50 necrotic fibers, individual or smaller groups of necrotic fibers are also seen   (MGI Ref ID J:27793)
  • calcified muscle
    • dystrophic calcification is seen ion areas of skeletal muscle necrosis   (MGI Ref ID J:27793)
  • dystrophic muscle   (MGI Ref ID J:144746)
  • myositis
    • accompanies skeletal muscle fiber necrosis   (MGI Ref ID J:27793)
  • immune system phenotype
  • myositis
    • accompanies skeletal muscle fiber necrosis   (MGI Ref ID J:27793)
  • hearing/vestibular/ear phenotype
  • abnormal auditory brainstem response waveform shape
    • prolonged I-IV interpeak latencies   (MGI Ref ID J:27793)
    • decreased wave IV amplitude   (MGI Ref ID J:27793)
    • mean wave IV threshold increased   (MGI Ref ID J:27793)
  • sensorineural hearing loss   (MGI Ref ID J:27793)
  • cardiovascular system phenotype
  • abnormal myocardium layer morphology
    • focal interstitial myocardial collagen deposition is seen at 10 months of age, indicating cardiac remodeling that occurs following myocardial damage   (MGI Ref ID J:169951)
    • abnormal myocardial fiber morphology
      • mutants exhibit focal patches of cardiac myocyte membrane damage   (MGI Ref ID J:169951)
  • homeostasis/metabolism phenotype
  • abnormal enzyme/coenzyme activity
    • mice exhibit hypoglycosylation of almost all alpha-dystroglycan compared to in wild-type mice   (MGI Ref ID J:144746)
    • laminin-binding activity of alpha-dystroglycan is less than 5% of normal   (MGI Ref ID J:144746)
  • increased circulating creatine kinase level
    • affected animals have an elevated serum CK range compared to controls   (MGI Ref ID J:27793)
  • behavior/neurological phenotype
  • abnormal gait
    • variable severity at 3 weeks of age; some exhibit gait abnormalities and others could not be identified by this observation   (MGI Ref ID J:27793)
  • limb grasping
    • homozygous mice tightly adduct the hindlimbs and curl toes when suspended by the tail   (MGI Ref ID J:27793)
  • growth/size/body phenotype
  • decreased body size
    • variable severity at 3 weeks of age; some are clearly smaller and others could not be identified by size   (MGI Ref ID J:27793)
View Research Applications

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

Developmental Biology Research
Postnatal Lethality
      Homozygous

Neurobiology Research
Muscular Dystrophy
      Dystroglycanopathy
Myelination Defects
      peripheral neuropathy

Largemyd related

Neurobiology Research
Muscular Dystrophy
      Dystroglycanopathy

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Largemyd
Allele Name myodystrophy
Allele Type Spontaneous
Common Name(s) Largemyd; fg; froggy; myd;
Strain of OriginSTOCK Edn3
Gene Symbol and Name Large, like-glycosyltransferase
Chromosome 8
Gene Common Name(s) BPFD#36; MDC1D; MDDGA6; MDDGB6; Mbp-1; Mbp1; enervated; enr; fg; froggy; mKIAA0609; myd; myelin basic protein transgene; myodystrophy;
Molecular Note The mutation underlying the myodystrophy phenotype has been determined to be an intragenic deletion in the glycotransferase gene, Large. The deletion of exons 5-7 cause a frameshift and a premature stop codon before the first two catalytic domains. [MGI Ref ID J:69796]

Genotyping

Genotyping Information

Genotyping Protocols

Largemyd, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Largemyd related

Barresi R; Michele DE; Kanagawa M; Harper HA; Dovico SA; Satz JS; Moore SA; Zhang W; Schachter H; Dumanski JP; Cohn RD; Nishino I; Campbell KP. 2004. LARGE can functionally bypass alpha-dystroglycan glycosylation defects in distinct congenital muscular dystrophies. Nat Med 10(7):696-703. [PubMed: 15184894]  [MGI Ref ID J:91681]

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]

Court FA; Hewitt JE; Davies K; Patton BL; Uncini A; Wrabetz L; Feltri ML. 2009. A laminin-2, dystroglycan, utrophin axis is required for compartmentalization and elongation of myelin segments. J Neurosci 29(12):3908-19. [PubMed: 19321787]  [MGI Ref ID J:147273]

Dwyer CA; Baker E; Hu H; Matthews RT. 2012. RPTPzeta/phosphacan is abnormally glycosylated in a model of muscle-eye-brain disease lacking functional POMGnT1. Neuroscience 220:47-61. [PubMed: 22728091]  [MGI Ref ID J:192518]

Grewal PK; Holzfeind PJ; Bittner RE; Hewitt JE. 2001. Mutant glycosyltransferase and altered glycosylation of alpha-dystroglycan in the myodystrophy mouse. Nat Genet 28(2):151-4. [PubMed: 11381262]  [MGI Ref ID J:69796]

Groh S; Zong H; Goddeeris MM; Lebakken CS; Venzke D; Pessin JE; Campbell KP. 2009. Sarcoglycan complex: implications for metabolic defects in muscular dystrophies. J Biol Chem 284(29):19178-82. [PubMed: 19494113]  [MGI Ref ID J:152269]

Gumerson JD; Davis CS; Kabaeva ZT; Hayes JM; Brooks SV; Michele DE. 2013. Muscle-specific expression of LARGE restores neuromuscular transmission deficits in dystrophic LARGEmyd mice. Hum Mol Genet 22(4):757-68. [PubMed: 23222475]  [MGI Ref ID J:191192]

Han R; Kanagawa M; Yoshida-Moriguchi T; Rader EP; Ng RA; Michele DE; Muirhead DE; Kunz S; Moore SA; Iannaccone ST; Miyake K; McNeil PL; Mayer U; Oldstone MB; Faulkner JA; Campbell KP. 2009. Basal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycan. Proc Natl Acad Sci U S A 106(31):12573-9. [PubMed: 19633189]  [MGI Ref ID J:152005]

Herbst R; Iskratsch T; Unger E; Bittner RE. 2009. Aberrant development of neuromuscular junctions in glycosylation-defective Large(myd) mice. Neuromuscul Disord 19(5):366-78. [PubMed: 19346129]  [MGI Ref ID J:157142]

Holzfeind PJ; Grewal PK; Reitsamer HA; Kechvar J; Lassmann H; Hoeger H; Hewitt JE; Bittner RE. 2002. Skeletal, cardiac and tongue muscle pathology, defective retinal transmission, and neuronal migration defects in the Large(myd) mouse defines a natural model for glycosylation-deficient muscle - eye - brain disorders. Hum Mol Genet 11(21):2673-87. [PubMed: 12354792]  [MGI Ref ID J:79438]

Hu H; Li J; Zhang Z; Yu M. 2011. Pikachurin interaction with dystroglycan is diminished by defective O-mannosyl glycosylation in congenital muscular dystrophy models and rescued by LARGE overexpression. Neurosci Lett 489(1):10-5. [PubMed: 21129441]  [MGI Ref ID J:168704]

Kabaeva Z; Meekhof KE; Michele DE. 2011. Sarcolemma instability during mechanical activity in Largemyd cardiac myocytes with loss of dystroglycan extracellular matrix receptor function. Hum Mol Genet 20(17):3346-55. [PubMed: 21628317]  [MGI Ref ID J:174976]

Kanagawa M; Michele DE; Satz JS; Barresi R; Kusano H; Sasaki T; Timpl R; Henry MD; Campbell KP. 2005. Disruption of perlecan binding and matrix assembly by post-translational or genetic disruption of dystroglycan function. FEBS Lett 579(21):4792-6. [PubMed: 16098969]  [MGI Ref ID J:101333]

Kanagawa M; Nishimoto A; Chiyonobu T; Takeda S; Miyagoe-Suzuki Y; Wang F; Fujikake N; Taniguchi M; Lu Z; Tachikawa M; Nagai Y; Tashiro F; Miyazaki J; Tajima Y; Takeda S; Endo T; Kobayashi K; Campbell KP; Toda T. 2009. Residual laminin-binding activity and enhanced dystroglycan glycosylation by LARGE in novel model mice to dystroglycanopathy. Hum Mol Genet 18(4):621-31. [PubMed: 19017726]  [MGI Ref ID J:144746]

Kuga A; Kanagawa M; Sudo A; Chan YM; Tajiri M; Manya H; Kikkawa Y; Nomizu M; Kobayashi K; Endo T; Lu QL; Wada Y; Toda T. 2012. Absence of post-phosphoryl modification in dystroglycanopathy mouse models and wild-type tissues expressing non-laminin binding form of alpha-dystroglycan. J Biol Chem 287(12):9560-7. [PubMed: 22270369]  [MGI Ref ID J:183288]

Lane PW. 1969. Froggy (fg) renamed myd - myodystrophy Mouse News Lett 40:30.  [MGI Ref ID J:64437]

Lane PW. 1974. fg changed to myd. Mouse News Lett 50:43.  [MGI Ref ID J:14773]

Lane PW; Beamer TC; Myers DD. 1976. Myodystrophy, a new myopathy on chromosome 8 of the mouse. J Hered 67(3):135-8. [PubMed: 939913]  [MGI Ref ID J:5670]

Lee Y; Kameya S; Cox GA; Hsu J; Hicks W; Maddatu TP; Smith RS; Naggert JK; Peachey NS; Nishina PM. 2005. Ocular abnormalities in Large(myd) and Large(vls) mice, spontaneous models for muscle, eye, and brain diseases. Mol Cell Neurosci 30(2):160-72. [PubMed: 16111892]  [MGI Ref ID J:100214]

Levedakou EN; Chen XJ; Soliven B; Popko B. 2005. Disruption of the mouse Large gene in the enr and myd mutants results in nerve, muscle, and neuromuscular junction defects. Mol Cell Neurosci 28(4):757-69. [PubMed: 15797722]  [MGI Ref ID J:96939]

Li J; Yu M; Feng G; Hu H; Li X. 2011. Breaches of the pial basement membrane are associated with defective dentate gyrus development in mouse models of congenital muscular dystrophies. Neurosci Lett 505(1):19-24. [PubMed: 21970971]  [MGI Ref ID J:178536]

Marshall JL; Holmberg J; Chou E; Ocampo AC; Oh J; Lee J; Peter AK; Martin PT; Crosbie-Watson RH. 2012. Sarcospan-dependent Akt activation is required for utrophin expression and muscle regeneration. J Cell Biol 197(7):1009-27. [PubMed: 22734004]  [MGI Ref ID J:185346]

Martins PC; Ayub-Guerrieri D; Martins-Bach AB; Onofre-Oliveira P; Malheiros JM; Tannus A; de Sousa PL; Carlier PG; Vainzof M. 2013. Dmdmdx/Largemyd: a new mouse model of neuromuscular diseases useful for studying physiopathological mechanisms and testing therapies. Dis Model Mech 6(5):1167-74. [PubMed: 23798567]  [MGI Ref ID J:201690]

Mathews KD; Mills KA; Bailey HL; Schelper RL; Murray JC. 1995. Mouse myodystrophy (myd) mutation: refined mapping in an interval flanked by homology with distal human 4q. Muscle Nerve Suppl(2):S98-102. [PubMed: 7739634]  [MGI Ref ID J:26061]

Mathews KD; Rapisarda D; Bailey HL; Murray JC; Schelper RL; Smith R. 1995. Phenotypic and pathologic evaluation of the myd mouse. A candidate model for facioscapulohumeral dystrophy. J Neuropathol Exp Neurol 54(4):601-6. [PubMed: 7602333]  [MGI Ref ID J:27793]

Michele DE; Barresi R; Kanagawa M; Saito F; Cohn RD; Satz JS; Dollar J; Nishino I; Kelley RI; Somer H; Straub V; Mathews KD; Moore SA; Campbell KP. 2002. Post-translational disruption of dystroglycan-ligand interactions in congenital muscular dystrophies. Nature 418(6896):417-22. [PubMed: 12140558]  [MGI Ref ID J:86900]

Michele DE; Kabaeva Z; Davis SL; Weiss RM; Campbell KP. 2009. Dystroglycan matrix receptor function in cardiac myocytes is important for limiting activity-induced myocardial damage. Circ Res 105(10):984-93. [PubMed: 19797173]  [MGI Ref ID J:169951]

Mobley BA. 1985. Ca2+ capacity and uptake rate in skinned fibers of myodystrophic muscle. Exp Neurol 87(1):137-46. [PubMed: 3155690]  [MGI Ref ID J:7713]

Neymark MA; Kopacz SJ; Lee CP. 1980. Characterization of ATPase in sarcoplasmic reticulum from two strains of dystrophic mice. Muscle Nerve 3(4):316-25. [PubMed: 6447833]  [MGI Ref ID J:6379]

Nutting DF; MacPike AD; Meier H. 1980. The calcium content of various tissues from myodystrophic and dystrophic mice. J Hered 71:15-18.  [MGI Ref ID J:12034]

Qu Q; Crandall JE; Luo T; McCaffery PJ; Smith FI. 2006. Defects in tangential neuronal migration of pontine nuclei neurons in the Largemyd mouse are associated with stalled migration in the ventrolateral hindbrain. Eur J Neurosci 23(11):2877-86. [PubMed: 16819976]  [MGI Ref ID J:111600]

Rayburn HB; Peterson AC. 1978. Naked axons in myodystrophic mice. Brain Res 146(2):380-4. [PubMed: 647395]  [MGI Ref ID J:5974]

Reed PW; Mathews KD; Mills KA; Bloch RJ. 2004. The sarcolemma in the Large(myd) mouse. Muscle Nerve 30(5):585-95. [PubMed: 15389724]  [MGI Ref ID J:104941]

Rurak J; Noel G; Lui L; Joshi B; Moukhles H. 2007. Distribution of potassium ion and water permeable channels at perivascular glia in brain and retina of the Large(myd) mouse. J Neurochem 103(5):1940-53. [PubMed: 17803675]  [MGI Ref ID J:128697]

Stalnaker SH; Aoki K; Lim JM; Porterfield M; Liu M; Satz JS; Buskirk S; Xiong Y; Zhang P; Campbell KP; Hu H; Live D; Tiemeyer M; Wells L. 2011. Glycomic analyses of mouse models of congenital muscular dystrophy. J Biol Chem 286(24):21180-90. [PubMed: 21460210]  [MGI Ref ID J:173659]

Yoshida-Moriguchi T; Yu L; Stalnaker SH; Davis S; Kunz S; Madson M; Oldstone MB; Schachter H; Wells L; Campbell KP. 2010. O-mannosyl phosphorylation of alpha-dystroglycan is required for laminin binding. Science 327(5961):88-92. [PubMed: 20044576]  [MGI Ref ID J:155796]

Health & husbandry

Health & Colony Maintenance Information

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, mice heterozygous for the Largemyd mutation are bred to wildtype mice from the colony. When breeding MYD/Le-Largemyd heterozygotes together at The Jackson Laboratory Repository, MYD/Le-Largemyd homozygous animals were small and died early.
Mating SystemWild-type x Heterozygote         (Female x Male)   26-MAR-13
Heterozygote x Wild-type         (Female x Male)   26-MAR-13
Diet Information LabDiet® 5K52/5K67

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $232.00Female or MaleHeterozygous for Largemyd  
Price per Pair (US dollars $)Pair Genotype
$302.00Heterozygous for Largemyd x Wild-type for Largemyd  
$302.00Wild-type for Largemyd x Heterozygous for Largemyd  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1500 unique mouse models across a vast array of research areas. Breeding colonies provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. If a Repository strain is not immediately available, then within 2 to 3 business days, you will receive an estimated availability timeframe for your inquiry or order along with various delivery options. Repository strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping. We will note and try to accommodate requests for specific ages of Repository strains but cannot guarantee provision of these strains at specific ages. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, please let us know.

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $301.60Female or MaleHeterozygous for Largemyd  
Price per Pair (US dollars $)Pair Genotype
$392.60Heterozygous for Largemyd x Wild-type for Largemyd  
$392.60Wild-type for Largemyd x Heterozygous for Largemyd  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1500 unique mouse models across a vast array of research areas. Breeding colonies provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. If a Repository strain is not immediately available, then within 2 to 3 business days, you will receive an estimated availability timeframe for your inquiry or order along with various delivery options. Repository strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping. We will note and try to accommodate requests for specific ages of Repository strains but cannot guarantee provision of these strains at specific ages. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, please let us know.

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Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1500 unique mouse models across a vast array of research areas. Breeding colonies provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. If a Repository strain is not immediately available, then within 2 to 3 business days, you will receive an estimated availability timeframe for your inquiry or order along with various delivery options. Repository strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping. We will note and try to accommodate requests for specific ages of Repository strains but cannot guarantee provision of these strains at specific ages. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, please let us know.

Control Information

  Control
   Wild-type from the colony
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.


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The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
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JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.


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