About   Help   FAQ
Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Lama2dy-2J
dystrophia muscularis 2 Jackson
MGI:1856027
Summary 6 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Lama2dy-2J/Lama2dy-2J B6.WK-Lama2dy-2J MGI:3789335
hm2
Lama2dy-2J/Lama2dy-2J B6.WK-Lama2dy-2J/J MGI:3789189
hm3
Lama2dy-2J/Lama2dy-2J involves: C57BL/6 * FVB/N MGI:3522490
hm4
Lama2dy-2J/Lama2dy-2J involves: WK/ReJ MGI:3789298
cx5
Lama2dy-2J/Lama2dy-2J
Lama4tm1Ktry/Lama4tm1Ktry
involves: 129X1/SvJ * C57BL/6 * WK/ReJ MGI:3789288
cx6
Lama2dy-2J/Lama2dy-2J
Lama4tm1Ktry/Lama4tm1Ktry
Tg(ACTB-Lama5)1Jhm/0
involves: 129X1/SvJ * C57BL/6 * WK/ReJ MGI:3789290


Genotype
MGI:3789335
hm1
Allelic
Composition
Lama2dy-2J/Lama2dy-2J
Genetic
Background
B6.WK-Lama2dy-2J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lama2dy-2J mutation (1 available); any Lama2 mutation (104 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• unlike in wild-type mice, one third of Purkinje cells observed exhibit long term potentitation when evoked excitatory postsynaptic potentials are recorded

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
congenital merosin-deficient muscular dystrophy 1A DOID:0110636 OMIM:607855
J:97464




Genotype
MGI:3789189
hm2
Allelic
Composition
Lama2dy-2J/Lama2dy-2J
Genetic
Background
B6.WK-Lama2dy-2J/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lama2dy-2J mutation (1 available); any Lama2 mutation (104 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Lama2dy-2J/Lama2dy-2J mouse

muscle
• unlike in wild-type mice, muscles contain myofibers of different calibers
• mice retain a higher regenerative capacity than Lama2dy homozygotes but exhibit an increase in centrally located nuclei compared to controls

nervous system
• mice exhibit defective radial sorting of nerve roots in the spinal cord
• mice exhibit amyelinated axons of peripheral nerves and roots (J:98086)
• peripheral nerve roots in the spine exhibit severe hypomyelination (J:134367)
• despite adherence of Schwann cells to axons and extension of processes, myelination is not completed (J:134367)
• mice exhibit a variable defect in myelination compared to Lama2dy-7J homozygotes that exhibit a consistent hypo-myelination phenotype (J:134367)

behavior/neurological
• when suspended by their tail, mice exhibit retraction of hindlimbs towards body
• mice exhibit locomotive impairments when prodded to walk fast
• however, when not prodded mice move normally

craniofacial
• the posterior portion of the skull is slightly expanded
• skull width relative to skull length is increased

growth/size/body

skeleton
• the posterior portion of the skull is slightly expanded
• skull width relative to skull length is increased

cellular
• the basal lamina on myelinating Schawann cells contains gaps
• the basal lamina was absent from non- and pre-myelinating Schwann cells




Genotype
MGI:3522490
hm3
Allelic
Composition
Lama2dy-2J/Lama2dy-2J
Genetic
Background
involves: C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lama2dy-2J mutation (1 available); any Lama2 mutation (104 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• from 2-3 weeks of age, flexed hind legs to the trunk when lifted by the tail

muscle
• develop mild form of muscular dystrophy, though body weight, body size and life span were comparable to wild-type
• had muscle degeneration, muscle fiber necrosis and fibrosis, though to a lesser extent than in Lama2tm1Eeng homozygotes




Genotype
MGI:3789298
hm4
Allelic
Composition
Lama2dy-2J/Lama2dy-2J
Genetic
Background
involves: WK/ReJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lama2dy-2J mutation (1 available); any Lama2 mutation (104 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• unlike wild-type mice, mice clasp their hindlimbs and alternatively flex and extend their those and legs when suspended by their tails
• mild
• mice drag one hindlimb or the other when walking

muscle
• mild
• 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

reproductive system
N
• unlike Lama2dy homozygotes, mice reproduce




Genotype
MGI:3789288
cx5
Allelic
Composition
Lama2dy-2J/Lama2dy-2J
Lama4tm1Ktry/Lama4tm1Ktry
Genetic
Background
involves: 129X1/SvJ * C57BL/6 * WK/ReJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lama2dy-2J mutation (1 available); any Lama2 mutation (104 available)
Lama4tm1Ktry mutation (1 available); any Lama4 mutation (61 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Severe neuropathy in Lama2dy-2J/Lama2dy-2J Lama4tm1Ktry/Lama4tm1Ktry mice

mortality/aging
• mice do not survive past 3 weeks of age

nervous system
N
• radial sorting of nerve roots in the spinal cord is normal
• the number of Schwann cells is decreased
• peripheral nerves are translucent instead of opaque white due to myelination
• mice exhibit amyelinated axons in the limbs

behavior/neurological
• mice display dyskinesia by P14
• mice exhibit a splayed stance and strong tremors, halting movement, and retraction of all limbs when suspended

growth/size/body
• mice are 50% smaller than wild-type mice

cellular
• pre- and promyelinating Schwann cells lack basal lamina
• however, ensheathment and myelination of axons by Schwann cells is normal




Genotype
MGI:3789290
cx6
Allelic
Composition
Lama2dy-2J/Lama2dy-2J
Lama4tm1Ktry/Lama4tm1Ktry
Tg(ACTB-Lama5)1Jhm/0
Genetic
Background
involves: 129X1/SvJ * C57BL/6 * WK/ReJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lama2dy-2J mutation (1 available); any Lama2 mutation (104 available)
Lama4tm1Ktry mutation (1 available); any Lama4 mutation (61 available)
Tg(ACTB-Lama5)1Jhm mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
N
• sorting and myelination in distal nerves are improved compared to in Lama2dy-2J Lama4tm1Blp homozygotes

behavior/neurological
N
• tremors and dyskinesia observed in Lama2dy-2J Lama4tm1Blp homozygotes are suppressed

cellular
• promyelinating Schwann cells lack a basal lamina





Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
Citing These Resources
Funding Information
Warranty Disclaimer & Copyright Notice
Send questions and comments to User Support.
last database update
09/10/2019
MGI 6.14
The Jackson Laboratory