Strain Name:

B6.129P1-Lama2dy/J

Stock Number:

000631

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Availability:

Cryopreserved - Ready for recovery

Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Type Congenic; Mutant Strain; Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
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Additional information on Congenic nomenclature.
Specieslaboratory mouse
Background Strain C57BL/6J
Donor Strain 129P1/Re
GenerationN10
Generation Definitions

Appearance
black, affected
Related Genotype: a/a Lama2dy/Lama2dy

black, unaffected
Related Genotype: a/a Lama2dy/+ or +/?

Description
Mice homozygous for the dystrophia-muscularis spontaneous mutations (Lama2dy and Lama2dy-2J) are characterized by progressive weakness and paralysis beginning at about 3 1/2 weeks of age. The hindlimbs are affected first, later the axial and forelimb musculature. Death usually occurs before 6 months of age, and mutant mice are usually sterile. Skeletal muscle shows degenerative changes with proliferation of sarcolemmal nuclei, increase in amount of interstitial tissue, and size variation among individual muscle fibers. In the dorsal and ventral roots of the peripheral nerves, both spinal and cranial, Schwann cells fail to separate and ensheathe axons so that groups of closely apposed naked axons, normally seen only in early stages, persist into adulthood. In the rest of the PNS, the basement membrane of Schwann cells is interrupted by gaps, the internodal gap in the nodes of Ranvier is lengthened, and there is delayed onset of myelination with fewer myelinated axons and shorter internode length.

In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. This is the case for the strain above. It should be noted that the phenotype could vary from that originally described. We will modify the strain description if necessary as published results become available.

Control Information

  Control
   Heterozygote from the colony
   Untyped from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Lama2dy allele
000641   129P1/ReJ-Lama2dy/J
View Strains carrying   Lama2dy     (1 strain)

Strains carrying other alleles of Lama2
013786   B6.129S1(Cg)-Lama2tm1Eeng/J
000524   B6.WK-Lama2dy-2J/J
005635   C57BL/6J-Lama2dy-7J/J
003589   D.B/20Ei-Lama2dy-6J/J
View Strains carrying other alleles of Lama2     (4 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, Congenital Merosin-Deficient, 1A; MDC1A
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Lama2dy/Lama2dy

        B6.129P1-Lama2dy/J
  • hearing/vestibular/ear phenotype
  • abnormal bony labyrinth
    • spaces normally filled with perilymph and endolymph fluids are obliterated with a fibrotic debris   (MGI Ref ID J:101670)
  • abnormal inner ear vestibule morphology
    • mice exhibit a degeneration of vestibular structures   (MGI Ref ID J:101670)
    • absent vestibular hair cells   (MGI Ref ID J:101670)
  • abnormal organ of Corti morphology
    • the structural integrity of the organ of Corti is altered with supporting and hair cells not exhibiting their normal vertical height and a slight compression of the organ, especially at the apical half turn   (MGI Ref ID J:101670)
    • abnormal cochlear hair cell morphology
      • hair cells in the organ of Corti not exhibiting their normal vertical height   (MGI Ref ID J:101670)
      • absent cochlear hair cells   (MGI Ref ID J:101670)
    • abnormal organ of Corti supporting cell morphology
      • supporting not exhibiting their normal vertical height   (MGI Ref ID J:101670)
  • cochlear degeneration   (MGI Ref ID J:101670)
    • stria vascularis degeneration   (MGI Ref ID J:101670)
  • increased or absent threshold for auditory brainstem response
    • mice exhibit an increase in brainstem auditory threshold compared to wild-type mice   (MGI Ref ID J:101670)
  • behavior/neurological phenotype
  • abnormal motor capabilities/coordination/movement
    • mice exhibit impaired motor activity   (MGI Ref ID J:101670)
    • however, no circling or head twisting is observed   (MGI Ref ID J:101670)
    • abnormal motor coordination/ balance
      • mice stumble when walking   (MGI Ref ID J:5000)
  • nervous system phenotype
  • abnormal cochlear hair cell morphology
    • hair cells in the organ of Corti not exhibiting their normal vertical height   (MGI Ref ID J:101670)
    • absent cochlear hair cells   (MGI Ref ID J:101670)
  • abnormal cochlear nerve morphology
    • few if any nerve fibers remain in the ear   (MGI Ref ID J:101670)
  • absent vestibular hair cells   (MGI Ref ID J:101670)
  • decreased nerve conduction velocity   (MGI Ref ID J:5948)
  • craniofacial phenotype
  • abnormal cranium morphology
    • the posterior portion of the skull is slightly expanded while the medial portion is narrowed   (MGI Ref ID J:129632)
    • mice exhibit wider braincases, a slight flexion in the middle and rotated mandibles   (MGI Ref ID J:129632)
    • increased cranium width
      • skull width relative to skull length increase is more pronounced than in Lama2dy-2J homozygotes   (MGI Ref ID J:129632)
  • reproductive system phenotype
  • decreased testis weight   (MGI Ref ID J:5000)
  • growth/size/body phenotype
  • decreased body weight
    • drastic   (MGI Ref ID J:5000)
  • homeostasis/metabolism phenotype
  • increased circulating prolactin level   (MGI Ref ID J:5000)
  • muscle phenotype
  • muscle weakness   (MGI Ref ID J:101670)
  • endocrine/exocrine gland phenotype
  • decreased testis weight   (MGI Ref ID J:5000)
  • skeleton phenotype
  • abnormal cranium morphology
    • the posterior portion of the skull is slightly expanded while the medial portion is narrowed   (MGI Ref ID J:129632)
    • mice exhibit wider braincases, a slight flexion in the middle and rotated mandibles   (MGI Ref ID J:129632)
    • increased cranium width
      • skull width relative to skull length increase is more pronounced than in Lama2dy-2J homozygotes   (MGI Ref ID J:129632)

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

Lama2dy/Lama2dy

        involves: 129P1/Re * C57BL/6
  • muscle phenotype
  • abnormal muscle regeneration
    • mice exhibit a decreased regenerative capacity with a reduction of total fiber number and a decrease in centrally localized nuclei compared to in wild-type mice   (MGI Ref ID J:134367)
  • abnormal skeletal muscle fiber morphology
    • only a few necrotic fibers are found at 10 days of age but by 15 days of age there are necrotic fibers undergoing phagocytosis and fibers with internal nuclei and by 20 days of age there is an increase in both the number of necrotic and regenerating fibers and a variability in the muscle fiber size   (MGI Ref ID J:152525)
    • centrally nucleated skeletal muscle fibers   (MGI Ref ID J:152525)
    • decreased skeletal muscle fiber number
      • the total number of muscle fibers in the soleus is decreased after 15 days of age   (MGI Ref ID J:152525)
    • increased variability of skeletal muscle fiber size
      • mutant muscles contain myofibers of different calibers with increased fiber loss and the presence of fibrotic lesions   (MGI Ref ID J:134367)
      • evident by 15 days of age   (MGI Ref ID J:152525)
    • skeletal muscle fiber necrosis   (MGI Ref ID J:152525)
  • myopathy   (MGI Ref ID J:134367)
  • skeletal muscle interstitial fibrosis   (MGI Ref ID J:134367)
    • proliferation of interstitial tissue is found skeletal muscle by 20 days of age   (MGI Ref ID J:152525)
  • nervous system phenotype
  • abnormal myelination
    • peripheral nerve roots in the spine exhibit severe hypomyelination   (MGI Ref ID J:134367)
    • despite adherence of Schwann cells to axons and extension of processes, myelination is not completed   (MGI Ref ID J:134367)
  • other phenotype
  • abnormal basal lamina morphology
    • the basal lamina of muscles is discontinuous, sparse and enmeshed with collagen fibrils from the collapse of the reticular lamina   (MGI Ref ID J:134367)
    • the basal lamina of Schwann cells is sparse   (MGI Ref ID J:134367)
    • the basal lamina was absent from non-myelinating Schwann cells   (MGI Ref ID J:134367)

Lama2dy/Lama2dy

        129P1/Re
  • mortality/aging
  • premature death
    • most homozygotes die between 1 and 6 months of age   (MGI Ref ID J:13125)
  • muscle phenotype
  • abnormal sarcolemma morphology
    • unusual proliferation of sarcolemmal nuclei   (MGI Ref ID J:13125)
  • abnormal skeletal muscle fiber morphology
    • in contrast to wild-type, space between fibers is increased and an increase in interstitial tissue is observed   (MGI Ref ID J:13125)
    • in some cases, fat cells are found between fibers   (MGI Ref ID J:13125)
    • unusual proliferation of nuclei both within and between the fibers   (MGI Ref ID J:13125)
    • affected fibers appear rounded rather than polygonal in transverse section   (MGI Ref ID J:13125)
    • centrally nucleated skeletal muscle fibers
      • some fibers, although otherwise normal, contain long chains of centrally, rather than peripherally, located nuclei   (MGI Ref ID J:13125)
    • increased variability of skeletal muscle fiber size
      • individual fibers exhibit size variations   (MGI Ref ID J:13125)
  • dystrophic muscle
    • muscular atrophy proceeds from hind quarters to axial and forelimb musculature   (MGI Ref ID J:13125)
  • progressive muscle weakness
    • mild paralysis is first observed at 3.5 week and progresses to hindlimb dragging by 8 weeks   (MGI Ref ID J:13125)
    • eventually there is a complete loss of locomotor function and premature death   (MGI Ref ID J:13125)
  • skeleton phenotype
  • kyphosis
    • observed at 8 weeks   (MGI Ref ID J:13125)
  • behavior/neurological phenotype
  • abnormal sexual interaction
    • failure to mate putatively due to physical disability   (MGI Ref ID J:13125)
    • gonad morphology is normal   (MGI Ref ID J:13125)
  • ataxia
    • ataxia with occasional unilateral paresis is first observed at 3.5 weeks of age   (MGI Ref ID J:13125)
  • paresis
    • unilateral paresis begins at 3.5 weeks progressing to bilateral paresis   (MGI Ref ID J:13125)
    • paresis is accompanied by spasmodic flexion and flaccid extension in hindlimbs   (MGI Ref ID J:13125)
    • mild paralysis is first observed at 3.5 weeks and progresses to hindlimb dragging by 8 weeks   (MGI Ref ID J:13125)
  • growth/size/body phenotype
  • decreased body weight
    • by two weeks of age, body weight is less than wildtype   (MGI Ref ID J:13125)
    • weight difference continues through out lifespan   (MGI Ref ID J:13125)
    • cachexia
      • cachexia as well as thinning and ruffling of fur is observed by 8 weeks   (MGI Ref ID J:13125)

Lama2dy/Lama2dy

        129P1/ReJ-Lama2dy/J
  • nervous system phenotype
  • abnormal Schwann cell morphology
    • after P7, the number of Schwann cell nuclei increases   (MGI Ref ID J:12730)
  • abnormal axonal transport
    • transport of dopamine-beta-hydrolase in nerves is reduced compared to in wild-type mice   (MGI Ref ID J:5709)
  • abnormal spinal cord morphology
    • decreased weight   (MGI Ref ID J:7911)
  • decreased brain weight   (MGI Ref ID J:7911)
  • demyelination
    • mice exhibit a 36% decrease in myelinated nerves at the sciatic I level   (MGI Ref ID J:5331)
    • in the dorsal and ventral roots of the spinal and cranial peripheral nerves, Schwann cells fail to separate and ensheathe axons so that groups of closely apposed naked axons, normally seen only in early stages, persist into adulthood   (MGI Ref ID J:5331)
    • fetal axons of less than 0.5 um diameter lack myelin or Schwann cell ensheathment in the peripheral nervous system   (MGI Ref ID J:12730)
    • the onset of myelination is delayed compared to in wild-type mice   (MGI Ref ID J:12730)
    • the length of myelinated segments added to an existing population are shorter than in wild-type mice   (MGI Ref ID J:12730)
    • myelinated internodal lengths are shorter than in wild-type mice in the peripheral nervous system   (MGI Ref ID J:12730)
  • other phenotype
  • abnormal basement membrane morphology
    • the basement membrane of Schwann cells around myelinated axons is gapped whereas it is absent in amyelinated zones   (MGI Ref ID J:5565)
    • nodes exhibit unusually long segments bare of basement membrane   (MGI Ref ID J:5565)
    • the internodal gap in the basement membrane of peripheral nerves at the nodes of Ranvier is lengthened compared to in wild-type mice   (MGI Ref ID J:5866)
  • homeostasis/metabolism phenotype
  • abnormal lipid level
    • mice exhibit a reduction in total spinal cord lipid levels and specifically galactolipids and cerebroside sulfatide) compared to wild-type mice   (MGI Ref ID J:7911)
    • mice exhibit a decrease in cerebroside sulfatide in the brain compared to wild-type mice   (MGI Ref ID J:7911)
    • increased cholesterol level
      • total cholesterol levels in the spinal cord are higher than in wild-type mice   (MGI Ref ID J:7911)
  • growth/size/body phenotype
  • decreased body weight   (MGI Ref ID J:7911)

Lama2dy/Lama2dy

        involves: 129P1/ReJ
  • mortality/aging
  • premature death
    • only 66% of mice survive the 24 week experiment compared to 86% of C3tm1Hrc Lama2dy homozygotes   (MGI Ref ID J:102959)
  • muscle phenotype
  • abnormal muscle physiology
    • mice display hindlimb contractures   (MGI Ref ID J:102959)
    • muscle weakness
      • forelimb strength is decreased compared to in C3tm1Hrc Lama2dy homozygotes   (MGI Ref ID J:102959)
  • dystrophic muscle   (MGI Ref ID J:102959)
  • skeletal muscle interstitial fibrosis   (MGI Ref ID J:102959)
  • growth/size/body phenotype
  • postnatal growth retardation
    • compared to C3tm1Hrc Lama2dy homozygotes   (MGI Ref ID J:102959)
  • immune system phenotype
  • increased IgG level
    • mice exhibit increased deposition of IgG in muscles compared to in wild-type mice   (MGI Ref ID J:102959)
  • increased IgM level
    • mice exhibit increased deposition of IgM in muscles compared to in wild-type mice and C3tm1Hrc Lama2dy homozygotes   (MGI Ref ID J:102959)
  • skeleton phenotype
  • scoliosis
    • unlike C3tm1Hrc Lama2dy homozygotes   (MGI Ref ID J:102959)
  • hematopoietic system phenotype
  • increased IgG level
    • mice exhibit increased deposition of IgG in muscles compared to in wild-type mice   (MGI Ref ID J:102959)
  • increased IgM level
    • mice exhibit increased deposition of IgM in muscles compared to in wild-type mice and C3tm1Hrc Lama2dy homozygotes   (MGI Ref ID J:102959)
View Research Applications

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

Neurobiology Research
Muscular Dystrophy
      Congenital Merosin-Deficient type

Lama2dy related

Immunology, Inflammation and Autoimmunity Research
Immunodeficiency Associated with Other Defects

Neurobiology Research
Muscular Dystrophy
      Congenital Merosin-Deficient type
Myelination Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Lama2dy
Allele Name dystrophia muscularis
Allele Type Spontaneous
Common Name(s) dy;
Strain of Origin129P1/Re
Gene Symbol and Name Lama2, laminin, alpha 2
Chromosome 10
Gene Common Name(s) LAMM; dy; dystrophia muscularis; mer; merosin; nmf417;
General Note This mutation arose spontaneously in the 129/Re inbred strain at The Jackson Laboratory in 1951.
Molecular Note Analysis of Lama2 expression of dystrophic dy mice revealed a specific deficiency of this mRNA in skeletal muscle, cardiac muscle, and peripheral nerve. [MGI Ref ID J:18187] [MGI Ref ID J:44989]

Genotyping

Genotyping Information


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Meier H; Southard JL. 1970. Muscular dystrophy in the mouse caused by an allele at the dy-locus. Life Sci 9(3):137-44. [PubMed: 5434356]  [MGI Ref ID J:5151]

Sunada Y; Bernier SM; Utani A; Yamada Y; Campbell KP. 1995. Identification of a novel mutant transcript of laminin alpha 2 chain gene responsible for muscular dystrophy and dysmyelination in dy2J mice. Hum Mol Genet 4(6):1055-61. [PubMed: 7655459]  [MGI Ref ID J:25954]

Xu H; Wu XR; Wewer UM; Engvall E. 1994. Murine muscular dystrophy caused by a mutation in the laminin alpha 2 (Lama2) gene. Nat Genet 8(3):297-302. [PubMed: 7874173]  [MGI Ref ID J:21367]

Lama2dy related

Amador AG; Mayerhofer A; Parkening TA; Collins TJ; Bartke A. 1992. Differential effects of two alleles of the dy locus on the pituitary-testicular axis of mice. Rev Esp Fisiol 48(3):157-66. [PubMed: 1301630]  [MGI Ref ID J:5000]

Arahata K; Hayashi YK; Koga R; Goto K; Lee JH; Miyagoe Y; Ishii H; Tsukahara T; Takeda S; Woo M; Nonaka I; Matsuzaki T; Sugita H. 1993. Laminin in animal models for muscular dystrophy - defect of Laminin-M in skeletal and cardiac muscles and peripheral nerve of the homozygous dystrophic dy/dy mice Proc Jpn Acad 69 (Series B)(10):259-264.  [MGI Ref ID J:17255]

Belvindrah R; Hankel S; Walker J; Patton BL; Muller U. 2007. Beta1 integrins control the formation of cell chains in the adult rostral migratory stream. J Neurosci 27(10):2704-17. [PubMed: 17344408]  [MGI Ref ID J:120076]

Besse S; Allamand V; Vilquin JT; Li Z; Poirier C; Vignier N; Hori H; Guenet JL; Guicheney P. 2003. Spontaneous muscular dystrophy caused by a retrotransposal insertion in the mouse laminin alpha2 chain gene. Neuromuscul Disord 13(3):216-22. [PubMed: 12609503]  [MGI Ref ID J:102806]

Bourke DL; Ontell M. 1986. Modification of the phenotypic expression of murine dystrophy: a morphological study. Anat Rec 214(1):17-24. [PubMed: 3954056]  [MGI Ref ID J:8221]

Bradley WG; Jaros E; Jenkison M. 1977. The nodes of Ranvier in the nerves of mice with muscular dystrophy. J Neuropathol Exp Neurol 36(5):797-806. [PubMed: 894325]  [MGI Ref ID J:5866]

Bradley WG; Jenkison M. 1973. Abnormalities of peripheral nerves in murine muscular dystrophy. J Neurol Sci 18(2):227-47. [PubMed: 4120487]  [MGI Ref ID J:5331]

Brimijoin S; Jablecki C. 1976. Reduced axonal transport of dopamine-beta-hydroxylase activity in dystrophic mice: evidence for abnormality of adrenergic nerve cells. Exp Neurol 53(2):454-64. [PubMed: 61889]  [MGI Ref ID J:5709]

Bunge RP; Bunge MB; Okada E; Cornbrooks CJ. 1980. Abnormalities expressed in cultures prepared from peripheral nerve tissues of trembler and dystrophic mice. In: Neurological Mutations Affecting Myelination. Elsevier North Holland Biomedical Press, Amsterdam.  [MGI Ref ID J:30709]

Carbonetto S. 1977. Neuromuscular transmission in dystrophic mice. J Neurophysiol 40(4):836-43. [PubMed: 196056]  [MGI Ref ID J:5851]

Chun SJ; Rasband MN; Sidman RL; Habib AA; Vartanian T. 2003. Integrin-linked kinase is required for laminin-2-induced oligodendrocyte cell spreading and CNS myelination. J Cell Biol 163(2):397-408. [PubMed: 14581460]  [MGI Ref ID J:86333]

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 Ref ID J:103061]

Connolly AM; Keeling RM; Streif EM; Pestronk A; Mehta S. 2002. Complement 3 deficiency and oral prednisolone improve strength and prolong survival of laminin alpha2-deficient mice. J Neuroimmunol 127(1-2):80-7. [PubMed: 12044978]  [MGI Ref ID J:102959]

Cote PD; Moukhles H; Carbonetto S. 2002. Dystroglycan is not required for localization of dystrophin, syntrophin, and neuronal nitric-oxide synthase at the sarcolemma but regulates integrin alpha 7B expression and caveolin-3 distribution. J Biol Chem 277(7):4672-9. [PubMed: 11741881]  [MGI Ref ID J:74657]

Dubowitz DJ; Tyszka JM; Sewry CA; Moats RA; Scadeng M; Dubowitz V. 2000. High resolution magnetic resonance imaging of the brain in the dy/dy mouse with merosin-deficient congenital muscular dystrophy. Neuromuscul Disord 10(4-5):292-8. [PubMed: 10838257]  [MGI Ref ID J:103093]

Fox MA; Ho MS; Smyth N; Sanes JR. 2008. A synaptic nidogen: developmental regulation and role of nidogen-2 at the neuromuscular junction. Neural Dev 3:24. [PubMed: 18817539]  [MGI Ref ID J:160738]

Gawlik KI; Akerlund M; Carmignac V; Elamaa H; Durbeej M. 2010. Distinct roles for laminin globular domains in laminin alpha1 chain mediated rescue of murine laminin alpha2 chain deficiency. PLoS One 5(7):e11549. [PubMed: 20657839]  [MGI Ref ID J:163098]

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 Ref ID J:124551]

Hanft LM; Rybakova IN; Patel JR; Rafael-Fortney JA; Ervasti JM. 2006. Cytoplasmic gamma-actin contributes to a compensatory remodeling response in dystrophin-deficient muscle. Proc Natl Acad Sci U S A 103(14):5385-90. [PubMed: 16565216]  [MGI Ref ID J:108299]

Harris JB; Ribchester RR. 1978. Neuromuscular transmission is adequate in identified abnormal dystrophic muscle fibres. Nature 271(5643):362-4. [PubMed: 202877]  [MGI Ref ID J:5922]

Hiramatsu M; Kashimata M; Takayama F; Tsubakida K; Ri K; Minami N. 1992. Reduced level of epidermal growth factor in the skeletal muscle of mice with muscular dystrophy. Horm Metab Res 24(3):138-9. [PubMed: 1577403]  [MGI Ref ID J:770]

Hoffman HA; Grieshaber CK. 1977. Kidney catalase (Ce-2). Mouse News Lett 56:51.  [MGI Ref ID J:13709]

Jaros E; Bradley WG. 1979. Atypical axon-Schwann cell relationships in the common peroneal nerve of the dystrophic mouse: an ultrastructural study. Neuropathol Appl Neurobiol 5(2):133-47. [PubMed: 471185]  [MGI Ref ID J:6188]

Jaros E; Jenkison M. 1983. Defective differentiation of peripheral nerves in the dystrophic mouse. Brain Res 282(3):231-42. [PubMed: 6831246]  [MGI Ref ID J:12730]

Jones DC; Zelditch ML; Peake PL; German RZ. 2007. The effects of muscular dystrophy on the craniofacial shape of Mus musculus. J Anat 210(6):723-30. [PubMed: 17459142]  [MGI Ref ID J:129632]

Jucker M; Tian M; Norton DD; Sherman C; Kusiak JW. 1996. Laminin alpha 2 is a component of brain capillary basement membrane: reduced expression in dystrophic dy mice. Neuroscience 71(4):1153-61. [PubMed: 8684619]  [MGI Ref ID J:35188]

Lightfoot PS; German RZ. 1998. The effects of muscular dystrophy on craniofacial growth in mice: a study of heterochrony and ontogenetic allometry. J Morphol 235(1):1-16. [PubMed: 9397579]  [MGI Ref ID J:106266]

Lucas-Heron B. 1997. A drug inhibits the mitochondrial protease inducing calmitine deficiency in skeletal muscle of patients with Duchenne's muscular dystrophy and dy/dy dystrophic mice. Biochem Biophys Res Commun 232(2):559-61. [PubMed: 9125222]  [MGI Ref ID J:38962]

Madrid RE; Jaros E; Cullen MJ; Bradley WG. 1975. Genetically determined defect of Schwann cell basement membrane in dystrophic mouse. Nature 257(5524):319-21. [PubMed: 1161034]  [MGI Ref ID J:5565]

Matsushima Y; Kikuchi T; Kikuchi H; Ichihara N; Ishikawa A; Ishijima Y; Tachibana M. 2005. A new mouse model for infantile neuroaxonal dystrophy, inad mouse, maps to mouse chromosome 1. Mamm Genome 16(2):73-8. [PubMed: 15859351]  [MGI Ref ID J:97670]

McLane JA; McClure WO. 1977. Rapid axoplasmic transport in dystrophic mice. J Neurochem 29(5):865-72. [PubMed: 73571]  [MGI Ref ID J:5906]

Meinen S; Barzaghi P; Lin S; Lochmuller H; Ruegg MA. 2007. Linker molecules between laminins and dystroglycan ameliorate laminin-alpha2-deficient muscular dystrophy at all disease stages. J Cell Biol 176(7):979-93. [PubMed: 17389231]  [MGI Ref ID J:178132]

Michelson AM; Russell ES; Harman PJ. 1955. Dystrophia muscularis: a hereditary primary myopathy in the house mouse. Proc Natl Acad Sci U S A 41(12):1079-1084. [PubMed: 16589799]  [MGI Ref ID J:13125]

Montgomery A. 1975. Parabiotic reinnervation in normal and dystrophic mice. Part 1. Muscle weight and physiological studies. J Neurol Sci 26(3):401-23. [PubMed: 1185240]  [MGI Ref ID J:5580]

Moral-Naranjo MT; Cabezas-Herrera J; Vidal CJ; Campoy FJ. 2002. Muscular dystrophy with laminin deficiency decreases the content of butyrylcholinesterase tetramers in sciatic nerves of Lama2dy mice. Neurosci Lett 331(3):155-8. [PubMed: 12383920]  [MGI Ref ID J:108039]

Moral-Naranjo MT; Campoy FJ; Cabezas-Herrera J; Vidal CJ. 1999. Increased butyrylcholinesterase levels in microsomal membranes of dystrophic Lama2dy mouse muscle. J Neurochem 73(3):1138-44. [PubMed: 10461905]  [MGI Ref ID J:56933]

Moral-Naranjo MT; Montenegro MF; Munoz-Delgado E; Campoy FJ; Vidal CJ. 2010. The levels of both lipid rafts and raft-located acetylcholinesterase dimers increase in muscle of mice with muscular dystrophy by merosin deficiency. Biochim Biophys Acta 1802(9):754-64. [PubMed: 20670915]  [MGI Ref ID J:165446]

Mukasa T; Momoi T; Momoi MY. 1999. Activation of caspase-3 apoptotic pathways in skeletal muscle fibers in laminin alpha2-deficient mice. Biochem Biophys Res Commun 260(1):139-42. [PubMed: 10381357]  [MGI Ref ID J:55877]

Neerunjun JS; Dubowitz V. 1977. Regeneration of muscles transplanted between normal and dystrophic mice: a quantitative study of early transplants. J Anat 124(2):459-67. [PubMed: 591440]  [MGI Ref ID J:5904]

Nieto-Ceron S; del Campo LF; Munoz-Delgado E; Vidal CJ; Campoy FJ. 2005. Muscular dystrophy by merosin deficiency decreases acetylcholinesterase activity in thymus of Lama2dy mice. J Neurochem 95(4):1035-46. [PubMed: 16135075]  [MGI Ref ID J:102887]

Ohlendieck K; Campbell KP. 1991. Dystrophin-associated proteins are greatly reduced in skeletal muscle from mdx mice. J Cell Biol 115(6):1685-94. [PubMed: 1757468]  [MGI Ref ID J:1875]

Ontell MP; Hughes D; Hauschka SD; Ontell M. 1992. Transient neonatal denervation alters the proliferative capacity of myosatellite cells in dystrophic (129ReJdy/dy) muscle. J Neurobiol 23(4):407-19. [PubMed: 1634888]  [MGI Ref ID J:1640]

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 Ref ID J:77657]

Patton BL; Wang B; Tarumi YS; Seburn KL; Burgess RW. 2008. A single point mutation in the LN domain of LAMA2 causes muscular dystrophy and peripheral amyelination. J Cell Sci 121(Pt 10):1593-604. [PubMed: 18430779]  [MGI Ref ID J:134367]

Peterson AC; Bray GM. 1984. Normal basal laminas are realized on dystrophic Schwann cells in dystrophic in equilibrium shiverer chimera nerves. J Cell Biol 99(5):1831-7. [PubMed: 6490723]  [MGI Ref ID J:7618]

Pillers DA; Kempton JB; Duncan NM; Pang J; Dwinnell SJ; Trune DR. 2002. Hearing loss in the laminin-deficient dy mouse model of congenital muscular dystrophy. Mol Genet Metab 76(3):217-24. [PubMed: 12126936]  [MGI Ref ID J:101670]

Qiao C; Li J; Zhu T; Draviam R; Watkins S; Ye X; Chen C; Li J; Xiao X. 2005. Amelioration of laminin-alpha2-deficient congenital muscular dystrophy by somatic gene transfer of miniagrin. Proc Natl Acad Sci U S A 102(34):11999-2004. [PubMed: 16103356]  [MGI Ref ID J:101148]

Radakovits R; Barros CS; Belvindrah R; Patton B; Muller U. 2009. Regulation of radial glial survival by signals from the meninges. J Neurosci 29(24):7694-705. [PubMed: 19535581]  [MGI Ref ID J:150125]

Rasminsky M; Kearney RE; Aguayo AJ; Bray GM. 1978. Conduction of nervous impulses in spinal roots and peripheral nerves of dystrophic mice. Brain Res 143(1):71-85. [PubMed: 630405]  [MGI Ref ID J:5948]

Relan NK; Yang Y; Beqaj S; Miner JH; Schuger L. 1999. Cell elongation induces laminin alpha2 chain expression in mouse embryonic mesenchymal cells: role in visceral myogenesis. J Cell Biol 147(6):1341-50. [PubMed: 10601345]  [MGI Ref ID J:58931]

Relucio J; Tzvetanova ID; Ao W; Lindquist S; Colognato H. 2009. Laminin alters fyn regulatory mechanisms and promotes oligodendrocyte development. J Neurosci 29(38):11794-806. [PubMed: 19776266]  [MGI Ref ID J:153045]

Sakuma K; Watanabe K; Totsuka T; Kato K. 1998. Pathological changes in levels of three small stress proteins, alphaB crystallin, HSP 27 and p20, in the hindlimb muscles of dy mouse. Biochim Biophys Acta 1406(2):162-8. [PubMed: 9573353]  [MGI Ref ID J:47346]

Sewry CA; Uziyel Y; Torelli S; Buchanan S; Sorokin L; Cohen J ; Watt DJ. 1998. Differential labelling of laminin alpha 2 in muscle and neural tissue of dy/dy mice: are there isoforms of the laminin alpha 2 chain? Neuropathol Appl Neurobiol 24(1):66-72. [PubMed: 9549731]  [MGI Ref ID J:46894]

Sorokin LM; Maley MA; Moch H; von der Mark H; von der Mark K; Cadalbert L; Karosi S; Davies MJ; McGeachie JK; Grounds MD. 2000. Laminin alpha4 and integrin alpha6 are upregulated in regenerating dy/dy skeletal muscle: comparative expression of laminin and integrin isoforms in muscles regenerating after crush injury. Exp Cell Res 256(2):500-14. [PubMed: 10772822]  [MGI Ref ID J:61770]

Sunada Y; Bernier SM; Kozak CA; Yamada Y; Campbell KP. 1994. Deficiency of merosin in dystrophic dy mice and genetic linkage of laminin M chain gene to dy locus. J Biol Chem 269(19):13729-32. [PubMed: 8188645]  [MGI Ref ID J:18187]

Tiger CF; Gullberg D. 1997. Abscence of laminin alpha1 chain in the skeletal muscle of dystrophic dy/dy mice. Muscle Nerve 20(12):1515-24. [PubMed: 9390664]  [MGI Ref ID J:44989]

Tsuji S; Matsushita H. 1985. Evidence on hypomyelination of central nervous system in murine muscular dystrophy. J Neurol Sci 68(2-3):175-84. [PubMed: 2989438]  [MGI Ref ID J:7911]

Vilquin JT; Guerette B; Puymirat J; Yaffe D; Tome FM; Fardeau M; Fiszman M; Schwartz K; Tremblay JP. 1999. Myoblast transplantations lead to the expression of the laminin alpha 2 chain in normal and dystrophic (dy/dy) mouse muscles. Gene Ther 6(5):792-800. [PubMed: 10505103]  [MGI Ref ID J:55289]

Vilquin JT; Kinoshita I; Roy B; Goulet M; Engvall E; Tome F; Fardeau M; Tremblay JP. 1996. Partial laminin alpha2 chain restoration in alpha2 chain-deficient dy/dy mouse by primary muscle cell culture transplantation. J Cell Biol 133(1):185-97. [PubMed: 8601607]  [MGI Ref ID J:32227]

Woo M; Tanabe Y; Ishii H; Nonaka I; Yokoyama M; Esaki K. 1987. Muscle fiber growth and necrosis in dystrophic muscles: a comparative study between dy and mdx mice. J Neurol Sci 82(1-3):111-22. [PubMed: 3440862]  [MGI Ref ID J:152525]

Xu H; Christmas P; Wu XR; Wewer UM; Engvall E. 1994. Defective muscle basement membrane and lack of M-laminin in the dystrophic dy/dy mouse. Proc Natl Acad Sci U S A 91(12):5572-6. [PubMed: 8202529]  [MGI Ref ID J:18709]

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 Ref ID J:62398]

van Lunteren E; Moyer M. 2003. Sternohyoid muscle fatigue properties of dy/dy dystrophic mice, an animal model of merosin-deficient congenital muscular dystrophy. Pediatr Res 54(4):547-53. [PubMed: 12840158]  [MGI Ref ID J:102296]

van Lunteren E; Moyer M; Leahy P. 2006. Gene expression profiling of diaphragm muscle in alpha2-laminin (merosin)-deficient dy/dy dystrophic mice. Physiol Genomics 25(1):85-95. [PubMed: 16368874]  [MGI Ref ID J:108348]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

Colony Maintenance

Diet Information LabDiet® 5K52/5K67

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


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

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $3175.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery of Strains Needing Progeny Testing
    At least two untested males and two untested females (two pairs) will be recovered (eight or more mice is typical). The total number of animals provided, their gender and genotype will vary. Untested animals typically are available to ship between 13 and 16 weeks from the date of your order. If the first recovery attempt is unsuccessful, a second recovery will be done, extending the overall recovery time to approximately 25 weeks. Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals to you for progeny testing at your facility. If you perform the progeny testing, there is NO guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation.
    Please note that identified pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation.

    Please contact Customer Service for more information on the cost of progeny testing for a strain: Tel: 1-800-422-6423 or 1-207-288-5845 (from any location). The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
    Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $4127.50
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery of Strains Needing Progeny Testing
    At least two untested males and two untested females (two pairs) will be recovered (eight or more mice is typical). The total number of animals provided, their gender and genotype will vary. Untested animals typically are available to ship between 13 and 16 weeks from the date of your order. If the first recovery attempt is unsuccessful, a second recovery will be done, extending the overall recovery time to approximately 25 weeks. Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals to you for progeny testing at your facility. If you perform the progeny testing, there is NO guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation.
    Please note that identified pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation.

    Please contact Customer Service for more information on the cost of progeny testing for a strain: Tel: 1-800-422-6423 or 1-207-288-5845 (from any location). The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
    Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Control Information

  Control
   Heterozygote from the colony
   Untyped from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

Payment Terms and Conditions

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.


See Terms of Use tab for General Terms and Conditions


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.
Ordering Information
JAX® Mice
Surgical and Preconditioning Services
JAX® Services
Customer Services and Support
Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
Technical Support Email Form

Terms of Use

Terms of Use


General Terms and Conditions


Contact information

General inquiries regarding Terms of Use

Contracts Administration

phone:207-288-6470

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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