Description

Strain Information

Type Congenic; Mutant Strain; Spontaneous Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Mating System Ovarian Transplant-Cross-Intercross         (Female x Male)   01-MAR-06 Species laboratory mouse Background Strain C57BL/6J Donor Strain WK/Re Generation N22+N22F6 (01-FEB-11) Generation Definitions

Appearance
black, affected
Related Genotype: a/a Lama2^dy-2J/Lama2^dy-2J

black, unaffected
Related Genotype: a/a Lama2^dy-2J/+ or a/a +/?

Description
Mice homozygous for the dystrophia-muscularis spontaneous mutations (Lama2^dy and Lama2^dy-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 other alleles of Lama2

000641	129P1/ReJ-Lama2dy/J
000631	B6.129P1-Lama2dy/J
013786	B6.129S1(Cg)-Lama2tm1Eeng/J
005635	C57BL/6J-Lama2dy-7J/J
003589	D.B/20Ei-Lama2dy-6J/J

View Strains carrying other alleles of Lama2     (5 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

Lama2^dy-2J/Lama2^dy-2J

        B6.WK-Lama2^dy-2J/J

• muscle phenotype
• abnormal muscle regeneration
  • mice retain a higher regenerative capacity than Lama2^dy homozygotes but exhibit an increase in centrally located nuclei compared to controls   (MGI Ref ID J:134367)
• increased variability of skeletal muscle fiber size
  • unlike in wild-type mice, muscles contain myofibers of different calibers   (MGI Ref ID J:134367)
• 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)
  • mice exhibit a variable defect in myelination compared to Lama2^dy-7J homozygotes that exhibit a consistent hypo-myelination phenotype   (MGI Ref ID J:134367)
  • mice exhibit amyelinated axons of peripheral nerves and roots   (MGI Ref ID J:98086)
• abnormal nervous system morphology
  • mice exhibit defective radial sorting of nerve roots in the spinal cord   (MGI Ref ID J:98086)
• behavior/neurological phenotype
• abnormal motor coordination/ balance
  • mice exhibit locomotive impairments when prodded to walk fast   (MGI Ref ID J:5000)
  • however, when not prodded mice move normally   (MGI Ref ID J:5000)
• limb grasping
  • when suspended by their tail, mice exhibit retraction of hindlimbs towards body   (MGI Ref ID J:98086)
• craniofacial phenotype
• abnormal cranium morphology
  • the posterior portion of the skull is slightly expanded   (MGI Ref ID J:129632)
• • increased cranium width
    • skull width relative to skull length is increased   (MGI Ref ID J:129632)
• other phenotype
• abnormal basal lamina morphology
  • the basal lamina on myelinating Schawann cells contains gaps   (MGI Ref ID J:134367)
  • the basal lamina was absent from non- and pre-myelinating Schwann cells   (MGI Ref ID J:134367)
• growth/size phenotype
• decreased body weight   (MGI Ref ID J:5000)
• skeleton phenotype
• abnormal cranium morphology
  • the posterior portion of the skull is slightly expanded   (MGI Ref ID J:129632)
• • increased cranium width
    • skull width relative to skull length is increased   (MGI Ref ID J:129632)

Lama2^dy-2J/Lama2^dy-2J

        B6.WK-Lama2^dy-2J

• nervous system phenotype
• enhanced long term potentiation
  • unlike in wild-type mice, one third of Purkinje cells observed exhibit long term potentitation when evoked excitatory postsynaptic potentials are recorded   (MGI Ref ID J:97464)
• reduced long term depression   (MGI Ref ID J:97464)

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

Lama2^dy-2J/Lama2^dy-2J

        involves: C57BL/6 * FVB/N

• behavior/neurological phenotype
• limb grasping
  • from 2-3 weeks of age, flexed hind legs to the trunk when lifted by the tail   (MGI Ref ID J:49435)
• muscle phenotype
• dystrophic muscle
  • develop mild form of muscular dystrophy, though body weight, body size and life span were comparable to wild-type   (MGI Ref ID J:49435)
  • had muscle degeneration, muscle fiber necrosis and fibrosis, though to a lesser extent than in Lama2^tm1Eeng homozygotes   (MGI Ref ID J:49435)

Lama2^dy-2J/Lama2^dy-2J

        involves: WK/ReJ

• behavior/neurological phenotype
• hindlimb paralysis
  • mice drag one hindlimb or the other when walking   (MGI Ref ID J:5151)
• limb grasping
  • unlike wild-type mice, mice clasp their hindlimbs and alternatively flex and extend their those and legs when suspended by their tails   (MGI Ref ID J:5151)
• opisthotonus
  • mild   (MGI Ref ID J:5151)
• muscle phenotype
• dystrophic muscle
  • mice exhibit focal dystrophic lesions such as loss of striation, coagulation necrosis, regenerative activity, variation in fiber size, fibrotic replacement of fibers or increases in connective tissue surrounding muscle fibers, and internal rowing of nuclei   (MGI Ref ID J:5151)
• opisthotonus
  • mild   (MGI Ref ID J:5151)
• reproductive system phenotype
• *normal* reproductive system phenotype
  • unlike Lama2^dy homozygotes, mice reproduce   (MGI Ref ID J:5151)

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

Lama2^dy-2J 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-2J
Allele Name		dystrophia muscularis 2 Jackson
Allele Type		Spontaneous
Common Name(s)		2J; dy2J;
Strain of Origin		WK/ReJ
Gene Symbol and Name		Lama2, laminin, alpha 2
Chromosome		10
Gene Common Name(s)		LAMM; dy; dystrophia muscularis; mer; merosin; nmf417;
Molecular Note		The G to A mutation in a splice site consensus sequence causes abnormal splicing and expression of multiple mRNAs. One mRNA is translated into an alpha 2 polypeptide with a deletion in domain VI. [MGI Ref ID J:21367] [MGI Ref ID J:25954]

Genotyping

Genotyping Information

Genotyping Protocols

Lama2^dy-2J, Pyrosequencing

Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Kuang W; Xu H; Vachon PH; Liu L; Loechel F; Wewer UM; Engvall E. 1998. Merosin-deficient congenital muscular dystrophy. Partial genetic correction in two mouse models. J Clin Invest 102(4):844-52. [PubMed: 9710454]  [MGI Ref ID J:49435]

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]

Additional References

Previtali SC; Nodari A; Taveggia C; Pardini C; Dina G; Villa A; Wrabetz L; Quattrini A; Feltri ML. 2003. Expression of laminin receptors in schwann cell differentiation: evidence for distinct roles. J Neurosci 23(13):5520-30. [PubMed: 12843252]  [MGI Ref ID J:84372]

Lama2^dy-2J 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]

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

Anderson JL; Head SI; Morley JW. 2005. Synaptic plasticity in the dy(2J) mouse model of laminin alpha2-deficient congenital muscular dystrophy. Brain Res 1042(1):23-8. [PubMed: 15823249]  [MGI Ref ID J:97464]

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 Ref ID J:186918]

Colognato H; Yurchenco PD. 1999. The laminin alpha2 expressed by dystrophic dy(2J) mice is defective in its ability to form polymers. Curr Biol 9(22):1327-30. [PubMed: 10574769]  [MGI Ref ID J:58529]

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]

Head SI; Bakker AJ; Liangas G. 2004. EDL and soleus muscles of the C57BL6J/dy2j laminin-alpha 2-deficient dystrophic mouse are not vulnerable to eccentric contractions. Exp Physiol 89(5):531-9. [PubMed: 15184359]  [MGI Ref ID J:105416]

Jannapureddy SR; Patel ND; Hwang W; Boriek AM. 2003. Genetic Models in Applied Physiology. Merosin deficiency leads to alterations in passive and active skeletal muscle mechanics. J Appl Physiol 94(6):2524-33; discussion 2523. [PubMed: 12736195]  [MGI Ref ID J:103017]

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]

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]

Macpike AD; Meier H. 1976. Comparison of dy and dy2J, two alleles expressing forms of muscular dystrophy in the mouse. Proc Soc Exp Biol Med 151(4):670-2. [PubMed: 1265049]  [MGI Ref ID J:5636]

Miner JH; Li C; Patton BL. 2004. Laminins alpha2 and alpha4 in pancreatic acinar basement membranes are required for basal receptor localization. J Histochem Cytochem 52(2):153-6. [PubMed: 14729866]  [MGI Ref ID J:121049]

Montgomery A; Swenarchuk L. 1977. Dystrophic mice show age related muscle fiber and myelinated axon losses. Nature 267(5607):167-168. [PubMed: 16073433]  [MGI Ref ID J:12011]

Nevo Y; Aga-Mizrachi S; Elmakayes E; Yanay N; Ettinger K; Elbaz M; Brunschwig Z; Dadush O; Elad-Sfadia G; Haklai R; Kloog Y; Chapman J; Reif S. 2011. The Ras antagonist, farnesylthiosalicylic acid (FTS), decreases fibrosis and improves muscle strength in dy/dy mouse model of muscular dystrophy. PLoS One 6(3):e18049. [PubMed: 21445359]  [MGI Ref ID J:171671]

Occhi S; Zambroni D; Del Carro U; Amadio S; Sirkowski EE; Scherer SS; Campbell KP; Moore SA; Chen ZL; Strickland S; Di Muzio A; Uncini A; Wrabetz L; Feltri ML. 2005. Both laminin and Schwann cell dystroglycan are necessary for proper clustering of sodium channels at nodes of ranvier. J Neurosci 25(41):9418-27. [PubMed: 16221851]  [MGI Ref ID J:101621]

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]

Perkins CS; Bray GM; Aguayo AJ. 1981. Ongoing block of Schwann cell differentiation and deployment in dystrophic mouse spinal roots. Brain Res 227(2):213-20. [PubMed: 7225891]  [MGI Ref ID J:12727]

Spector I; Zilberstein Y; Lavy A; Genin O; Barzilai-Tutsch H; Bodanovsky A; Halevy O; Pines M. 2013. The involvement of collagen triple helix repeat containing 1 in muscular dystrophies. Am J Pathol 182(3):905-16. [PubMed: 23274062]  [MGI Ref ID J:193271]

Weinberg HJ; Spencer PS; Raine CS. 1975. Aberrant PNS development in dystrophic mice. Brain Res 88(3):532-7. [PubMed: 1139295]  [MGI Ref ID J:5546]

Yang D; Bierman J; Tarumi YS; Zhong YP; Rangwala R; Proctor TM; Miyagoe-Suzuki Y; Takeda S; Miner JH; Sherman LS; Gold BG; Patton BL. 2005. Coordinate control of axon defasciculation and myelination by laminin-2 and -8. J Cell Biol 168(4):655-66. [PubMed: 15699217]  [MGI Ref ID J:98086]

Yurchenco PD; Cheng YS; Campbell K; Li S. 2004. Loss of basement membrane, receptor and cytoskeletal lattices in a laminin-deficient muscular dystrophy. J Cell Sci 117(Pt 5):735-42. [PubMed: 14734655]  [MGI Ref ID J:87908]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           FGB29

Colony Maintenance

Mating System	Ovarian Transplant-Cross-Intercross         (Female x Male)   01-MAR-06

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls

General Supply Notes

• View the complete collection of spontaneous mutants in the Mouse Mutant Resource.
• Genomic DNA is available for this strain from the Mouse DNA Resource.

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.

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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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