Strain Name:

MYD/Le-Os +/+ Largemyd/J

Stock Number:

000300

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Availability:

Cryopreserved - Ready for recovery

The semidominant oligosyndactlism (Os) mutation is maintained in repulsion with the spontaneous mutation myodystrophy(Largemyd). Myodystrophy (myd) is a spontaneous mutation in Large (like-glycosyltransferase), a glycosyltransferase involved in the glycosylation of alpha-dystroglycan. Mice homozygous for the mutation exhibit diffuse and progressive myopathy, as well as hearing loss and retinal abnormalities. This allele may be useful for studying congenital muscular dystrophy with brain and eye abnormalities (MDDGA6). Mice heterozygous for oligosyndactlism (Os) exhibit fused digits in all four feet and small kidney size.

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 Mutant Strain;
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Specieslaboratory mouse
GenerationN7 F28
Generation Definitions

Description
Large (like-glycosyltransferase) encodes a glycosyltransferase involved in the glycosylation of alpha-dystroglycan. Mutations in LARGE are associated with congenital muscular dystrophy with brain and eye abnormalities (MDDGA6). Mice homozygous for the myodystrophy (myd) allele exhibit a short, shuffling gait, kyphosis, diffuse and progressive myopathy, myositis, cardiomyopathy and a reduced lifespan (average 17.25 weeks). Skeletal muscle pathologies include variation in fiber size, loss of striation, centralized nuclei, calcification and nuclear rowing. Other characteristics include hearing loss, disorganized retinal layers, and axon demyelination in the peripheral nervous system.

Mice heterozygous for oligosyndactlism (Os) exhibit fused digits in all four feet, progressive diabetes, small kidney size and reduced numbers of kidney glomeruli. Homozygotes are embryonic lethal. The semidominant oligosyndactlism (Os) mutation is maintained in repulsion with the spontaneous mutation myodystrophy(Largemyd).

Development
Myodystrophy (Largemyd) arose spontaneously in 1963 at The Jackson Laboratory in the lethal spotting stock (LS/LeJ) which had been imported from University College, London in 1961. The first affected male was outcrossed to a C57BL/6J female. Matings of a homoygote times a heterozygote were carried out as often as possible or as heterozygous pairs. Close linkage was found on Chromosome 8 and oligosyndactylism (Os) was used as a marker. A Largemyd/+ male at F38 was crossed to a pintail (Pt), oligosyndactyly (Os) female of the ROP strain and after 3 sib matings an Os/+ was selected and this genotype was crossed 7 times to the +/+ members of the strain. At N7 an Os/+ was again crossed to a Largemyd/+ and the strain was maintained by sibling matings selecting the Os phenotype which was generally Os +/+ Largemyd. It was cryopreserved in 1981 by mating Os +/+ Largemyd males at N7F22 -F27 to non Os (+ +/+ ?) females.

Control Information

  Control
   Untyped from the colony
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Largemyd allele
000226   B6C3Fe a/a-Largemyd/J
018305   MYD/Le-Largemyd/J
View Strains carrying   Largemyd     (2 strains)

Strains carrying   Os allele
000566   B6.Cg-Os +/+ Cacna1atg-la/J
003523   B6.ROP/Le-Os/J
000125   B6By.Cg-Sox18Ra Pt Os/J
000267   ROP/GnLeJ
002503   ROP/Le-Os Ces1ca/+ Ces1ca/J
View Strains carrying   Os     (5 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
  • 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)
  • 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)

Os/Os

        involves: 101 * C3H
  • mortality/aging
  • complete embryonic lethality between implantation and placentation   (MGI Ref ID J:5017)
  • embryogenesis phenotype
  • decreased embryo size
    • noted as early as E4.5   (MGI Ref ID J:5768)
  • embryonic growth arrest
    • cells of the developing embryo appear abnormal between the 7th and 8th division with suggestion of mitotic dysfunction   (MGI Ref ID J:5017)
    • the most striking abnormalities are seen by the middle of the 4th day when many embryonic cells contain fragmented, pycnotic chromatin and lack nuclear membrane and nucleolus   (MGI Ref ID J:5017)
    • in culture, blastocysts show rapid degeneration of the inner cell mass compared with control embryos   (MGI Ref ID J:5768)
  • cellular phenotype
  • abnormal mitosis   (MGI Ref ID J:5768)
    • increased mitotic index
      • E4.5 embryos have an index nine times that of controls   (MGI Ref ID J:5768)
      • more than one third of cells contain mitotic figures   (MGI Ref ID J:5768)
  • growth/size/body phenotype
  • decreased embryo size
    • noted as early as E4.5   (MGI Ref ID J:5768)

Os/Os+

        involves: 101 * C3H
  • embryogenesis phenotype
  • abnormal limb bud morphology
    • retardation of the mesodermal growth in the preaxial area of the footplate of the forelimb buds is found at embryonic day 10 hour 16 and in the hindlimb buds at embryonic day 10 hour 21   (MGI Ref ID J:5107)
    • although normal at early embryonic day 11, by embryonic day 11 to 12 there are cytolytic changes, cellular degeneration, in the preaxial part of the footplate mesoderm, leading to coalescence of the second and third digital rudiments   (MGI Ref ID J:5107)
  • limbs/digits/tail phenotype
  • abnormal limb bud morphology
    • retardation of the mesodermal growth in the preaxial area of the footplate of the forelimb buds is found at embryonic day 10 hour 16 and in the hindlimb buds at embryonic day 10 hour 21   (MGI Ref ID J:5107)
    • although normal at early embryonic day 11, by embryonic day 11 to 12 there are cytolytic changes, cellular degeneration, in the preaxial part of the footplate mesoderm, leading to coalescence of the second and third digital rudiments   (MGI Ref ID J:5107)

Os/Os+

        involves: 101 * C3H * CBA/Gr
  • mortality/aging
  • prenatal lethality   (MGI Ref ID J:13049)
  • limbs/digits/tail phenotype
  • abnormal carpal bone morphology
    • ulnar end of the hamatum articulates with metacarpal 4, but does not reach metacarpal 5   (MGI Ref ID J:13049)
    • metacarpal 5 is in a fixed state of abduction   (MGI Ref ID J:13049)
    • fused carpal bones
      • extensive fusions in carpus   (MGI Ref ID J:13049)
  • abnormal foot plate morphology
    • beginning at E11 the preaxial border of the foot plate is flattened, displaying an ovoid rather than circular outline   (MGI Ref ID J:12942)
    • blastemata are crowded and small   (MGI Ref ID J:12942)
    • interdigital area between digits 2 and 3 is reduced by E13   (MGI Ref ID J:12942)
    • in most cases, digit 2 is formed closer to and may fuse to digit 3   (MGI Ref ID J:12942)
  • abnormal limb mesenchyme morphology
    • a reduction in the amount of mesenchyme in the preaxial area of the foot plate is observed by E13   (MGI Ref ID J:12942)
  • abnormal tarsal bone morphology
    • long axis of calcaneus and metararsalia are not parallel, as a result hindfeet point outward   (MGI Ref ID J:13049)
    • abnormal calcaneum morphology
      • calcaneus frequently lacks process trochlearis   (MGI Ref ID J:13049)
    • abnormal navicular morphology
      • naviculare is narrow as compared to wildtype   (MGI Ref ID J:13049)
    • fused tarsal bones
      • extensive and varied fusions in tarsus, which includes a solid fusion between talus and calcaneus   (MGI Ref ID J:13049)
      • fusion occurs between the naviculare and a composite of cuneiforme 3 and cuboideum in all animals   (MGI Ref ID J:13049)
  • oligodactyly
    • digits 2 and 3 are typically involved   (MGI Ref ID J:13049)
    • different digits in the same foot can be both polydactylous and oligodactylous   (MGI Ref ID J:13049)
    • digit loss arises by fusion of digits 2 and 3, however, digit 2 is often thinner than normal and may vanish without fusion to digit 3   (MGI Ref ID J:12942)
  • polydactyly
    • exhibited in the hindfeet of some animals   (MGI Ref ID J:13049)
    • different digits in the same foot can be both polydactylous and oligodactylous   (MGI Ref ID J:13049)
    • polysyndactyly
      • exhibited in some animals   (MGI Ref ID J:13049)
  • syndactyly
    • all four feet are affected, although the forefeet are less severely affected than the hindfeet   (MGI Ref ID J:13049)
    • syndactylism primarily involves digits 2 and 3   (MGI Ref ID J:13049)
    • nearly all animals exhibit osseous fusions of the bases of metacarpalia or metatarsalia 4 and 5   (MGI Ref ID J:13049)
    • some animals exhibit fusion of metatarsalia 1 and 2   (MGI Ref ID J:13049)
    • most fusions are secondary, only a few of the fusions are primary hard tissue in both embryo and adult   (MGI Ref ID J:13049)
    • in the tarsus, only the cuneiforme 3 and cuboideum fusion is primary   (MGI Ref ID J:13049)
    • hard tissue fusions start at the basal phalanges and spread distally   (MGI Ref ID J:12942)
    • all fusions between metacarpal and metatarsals are secondary   (MGI Ref ID J:12942)
    • polysyndactyly
      • exhibited in some animals   (MGI Ref ID J:13049)
  • skeleton phenotype
  • abnormal carpal bone morphology
    • ulnar end of the hamatum articulates with metacarpal 4, but does not reach metacarpal 5   (MGI Ref ID J:13049)
    • metacarpal 5 is in a fixed state of abduction   (MGI Ref ID J:13049)
    • fused carpal bones
      • extensive fusions in carpus   (MGI Ref ID J:13049)
  • abnormal tarsal bone morphology
    • long axis of calcaneus and metararsalia are not parallel, as a result hindfeet point outward   (MGI Ref ID J:13049)
    • abnormal calcaneum morphology
      • calcaneus frequently lacks process trochlearis   (MGI Ref ID J:13049)
    • abnormal navicular morphology
      • naviculare is narrow as compared to wildtype   (MGI Ref ID J:13049)
    • fused tarsal bones
      • extensive and varied fusions in tarsus, which includes a solid fusion between talus and calcaneus   (MGI Ref ID J:13049)
      • fusion occurs between the naviculare and a composite of cuneiforme 3 and cuboideum in all animals   (MGI Ref ID J:13049)
  • embryogenesis phenotype
  • abnormal embryonic tissue morphology
    • at E14 projections of digits 2 and 3 at the edge of the foot plate are closer together than wildtype and there is only a single basal phalanx common to both digits   (MGI Ref ID J:13049)
    • at E14-5 cuneiforme 3 and cuboideum have fused to a single element and are elongated in a diagonal direction rather than circular   (MGI Ref ID J:13049)
    • at E16 metacarpalia 4 and 5 are fused   (MGI Ref ID J:13049)
    • abnormal limb mesenchyme morphology
      • a reduction in the amount of mesenchyme in the preaxial area of the foot plate is observed by E13   (MGI Ref ID J:12942)

Os/Os+

        ROP/GnLeJ
  • renal/urinary system phenotype
  • abnormal kidney morphology
    • renal mass is reduced by 38% in comparison to wild-type   (MGI Ref ID J:3842)
    • abnormal kidney collecting duct principal cell morphology
      • the principal cell type in the collecting duct is hypertrophied, with the greatest degree of hypertrophy in the nephrectomized heterozygotes   (MGI Ref ID J:3842)
    • abnormal nephron morphology
      • hypertrophied nephrons in females at 3 months of age   (MGI Ref ID J:28323)
      • abnormal proximal convoluted tubule morphology
        • segments of proximal tubule, especially pars recta, exhibit hypertrophy   (MGI Ref ID J:3842)
      • abnormal renal glomerulus morphology
        • size of glomeruli is slightly increased in left kidney following unilateral nephrectomy as compared to control   (MGI Ref ID J:3842)
        • this mutation on a C57BL/6J background does not show significant glomerular histopathology   (MGI Ref ID J:102341)
        • in femalesat 3 weeks of age there is a 34% increase in mean cell number per glomerulus compared with controls   (MGI Ref ID J:28323)
        • bone marrow transplant from heterozygotes into wild-type coisognic hosts results in mesangial sclerosis and glomerular hypertrophy   (MGI Ref ID J:166810)
        • decreased renal glomerulus number
          • midtranverse sections from the left kidney indicate that glomeruli density is reduced by 50% in heterozygotes   (MGI Ref ID J:3842)
          • females assessed at 3 months of age have a 55% reduction in the mean glomerular number per kidney   (MGI Ref ID J:28323)
        • expanded mesangial matrix   (MGI Ref ID J:166810)
          • in females at 3 months of age mesangial sclerosis affects all glomeruli and is evenly distributed in the cortical and justaglomerular regions, with increased extracellular matrix including type IV collagen and tenascin, but no inflammatory cells are found   (MGI Ref ID J:28323)
        • glomerulonephritis
          • glomerular cross-sectional area in females assessed at 3 months of age is increased by 1.8 fold compared with controls, although the fraction of the cortex occupied by glomeruli remains the same between mutant and control   (MGI Ref ID J:28323)
        • glomerulosclerosis   (MGI Ref ID J:166810)
          • on the ROP background but not the C57BL/6J background at 5 months of age there is severe and diffuse glomerulosclerosis restricted to the mesangial regions, with a large excess of type IV collagen and tenascin in the mesangial areas   (MGI Ref ID J:32764)
      • renal tubule hypertrophy
        • diameters of the proximal convoluted and straight tubules are increased in size as compared to wild-type   (MGI Ref ID J:3842)
        • tubular epithelial cells are hypertrophied in both heterozyote and nephrectomized heterozygotes, however, the magnitude of hypertrophy is increased in unaltered mice   (MGI Ref ID J:3842)
    • decreased compensatory renal growth
      • in nephrectomized heterozygote males, compensatory kidney growth is reduced in comparison to controls   (MGI Ref ID J:3842)
    • decreased kidney weight
      • females at 3 months of age have a 24% reduction in kidney weight but no concomitant change in body or heart weight   (MGI Ref ID J:28323)
  • increased urine flow rate
    • rate of urine flow is increased in heterozygotes as compared to controls, however glomerular filtration rate is not affected   (MGI Ref ID J:3842)
    • excretion of creatinine, sodium and potassium is similar to control   (MGI Ref ID J:3842)
  • homeostasis/metabolism phenotype
  • increased blood urea nitrogen level
    • BUN levels are increased by almost 50% in both heterozyote and nephrectomized heterozygotes as compared to controls   (MGI Ref ID J:3842)
  • immune system phenotype
  • glomerulonephritis
    • glomerular cross-sectional area in females assessed at 3 months of age is increased by 1.8 fold compared with controls, although the fraction of the cortex occupied by glomeruli remains the same between mutant and control   (MGI Ref ID J:28323)

Os/Os+

        B6.ROP/Le-Os/J
  • renal/urinary system phenotype
  • abnormal renal glomerulus morphology
    • glomerular volume is increased 2-fold relative to controls at 3 months of age and 3-fold at 5 months of age but the glomerulosclerosis found on the ROP background is absent with only a minimal increase in extracellular matrix at 5 months of age   (MGI Ref ID J:32764)
    • the mean cell number per glomerulus is increased 21% relative to controls and the glomerular labeling index is increased 2.6 fold at 3 and 5 months of age   (MGI Ref ID J:32764)
    • decreased renal glomerulus number
      • mean glomerular number per kidney is reduced by 50% at 3 months of age   (MGI Ref ID J:32764)
    • expanded mesangial matrix
      • only a minimal increase in extracellular matrix is noted at 5 months of age   (MGI Ref ID J:32764)
  • decreased kidney weight
    • 34% reduction in kidney weight compared with controls at 3 months of age   (MGI Ref ID J:32764)
View Research Applications

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

Neurobiology Research
Muscular Dystrophy
      Dystroglycanopathy

Largemyd related

Neurobiology Research
Muscular Dystrophy
      Dystroglycanopathy

Os related

Developmental Biology Research
Skeletal Defects
      Oligodactyly

Internal/Organ Research
Kidney Defects
      diabetes insipidus

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]
 
Allele Symbol Os
Allele Name Os
Allele Type Radiation induced
Strain of Origin(101 x C3H)F1
Gene Symbol and Name Os, oligosyndactylism
Chromosome 8
Gene Common Name(s) 94-A; 94-K; PlmTgN(Pgk1)1Ddp; PlmTgN(Pgk1)2Ddp; postimplantation lethal mutation induced by Pgk1 transgene insertion-Dimitrina D. Pravtcheva 1; postimplantation lethal mutation induced by Pgk1 transgene insertion-Dimitrina D. Pravtcheva 2;
General Note Heterozygotes are affected on all four feet. Fusion usually occurs between the second and third digits and occasionally involves the fourth (J:13049). The muscles of the forearms and lower legs as well as of the feet show anomalous arrangements not necessarily correlated with the skeletal changes (J:12944). At 11 days of gestation the preaxial border of the limbs can be seen to be reduced (J:12942), and a histological examination at this time shows that there is a small amount of cellular degeneration in the preaxial part of the footplate mesoderm, leading to coalescence of the second and third digital rudiments (J:5107). Os /+ mice have a mild diabetes insipidus present at 5 weeks and increasing with age. In combination with one or more recessive modifying genes in the selected DI stock, Os/+ mice have a severe diabetes insipidus (J:12948). The cause of the diabetes is a 45% reduction in size of the kidneys with an 80% reduction in number of glomeruli. Compensatory hypertrophy of the nephrons is not sufficient to restore normal urine-concentrating ability (J:5127)(J:5128).
Molecular Note The oligosyndactylism mutation is due to a chromosomal inversion that has breakpoints approximately 10 Mb apart. One breakpoint appears to reside in the Anapc10 gene, and an aberrant transcript consisting of part of Anapc10 and an unrelated sequence is expressed at low levels. [MGI Ref ID J:81567] [MGI Ref ID J:95333]

Genotyping

Genotyping Information

Genotyping Protocols

Largemyd, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

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]

Pravtcheva DD; Wise TL. 1996. A transgene-induced mitotic arrest mutation in the mouse allelic with Oligosyndactylism. Genetics 144(4):1747-56. [PubMed: 8978060]  [MGI Ref ID J:38877]

Pravtcheva DD; Wise TL. 2001. Disruption of Apc10/Doc1 in three alleles of oligosyndactylism. Genomics 72(1):78-87. [PubMed: 11247669]  [MGI Ref ID J:81567]

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]

Goddeeris MM; Wu B; Venzke D; Yoshida-Moriguchi T; Saito F; Matsumura K; Moore SA; Campbell KP. 2013. LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy. Nature 503(7474):136-40. [PubMed: 24132234]  [MGI Ref ID J:206057]

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]

Os related

Cornacchia F; Fornoni A; Plati AR; Thomas A; Wang Y; Inverardi L; Striker LJ; Striker GE. 2001. Glomerulosclerosis is transmitted by bone marrow-derived mesangial cell progenitors. J Clin Invest 108(11):1649-56. [PubMed: 11733560]  [MGI Ref ID J:166810]

Elliot SJ; Karl M; Berho M; Potier M; Zheng F; Leclercq B; Striker GE; Striker LJ. 2003. Estrogen deficiency accelerates progression of glomerulosclerosis in susceptible mice. Am J Pathol 162(5):1441-8. [PubMed: 12707027]  [MGI Ref ID J:83190]

Esposito C; He CJ; Striker GE; Zalups RK; Striker LJ. 1999. Nature and severity of the glomerular response to nephron reduction is strain-dependent in mice. Am J Pathol 154(3):891-7. [PubMed: 10079267]  [MGI Ref ID J:53353]

Falconer DS; Latyszewski M; Isaacson JH. 1964. Diabetes insipidus associated with oligosyndactylism in the mouse. Genet Res 5:473-488.  [MGI Ref ID J:12948]

Gruneberg H. 1956. Genetical studies on the skeleton of the mouse. XVIII. Three genes for syndactylism. J Genet 54:113-145.  [MGI Ref ID J:13049]

Gruneberg H. 1961. Genetical studies on the skeleton of the mouse. XXVII. The development of oligosyndactylism. Genet Res 2:33-42.  [MGI Ref ID J:12942]

He C; Esposito C; Phillips C; Zalups RK; Henderson DA; Striker GE; Striker LJ. 1996. Dissociation of glomerular hypertrophy, cell proliferation, and glomerulosclerosis in mouse strains heterozygous for a mutation (Os) which induces a 50% reduction in nephron number. J Clin Invest 97(5):1242-9. [PubMed: 8636436]  [MGI Ref ID J:32764]

He C; Zalups RK; Henderson DA; Striker GE; Striker LJ. 1995. Molecular analysis of spontaneous glomerulosclerosis in Os/+ mice, a model with reduced nephron mass. Am J Physiol 269(2 Pt 2):F266-73. [PubMed: 7544540]  [MGI Ref ID J:28323]

Jarad G; Lakhe-Reddy S; Blatnik J; Koepke M; Khan S; El-Meanawy MA; O'Connor AS; Sedor JR; Schelling JR. 2004. Renal phenotype is exacerbated in Os and lpr double mutant mice. Kidney Int 66(3):1029-35. [PubMed: 15327396]  [MGI Ref ID J:102341]

Kadam KM. 1962. Genetical studies on the skeleton of the mouse. XXXI. The muscular anatomy of syndactylism and oligosyndactylism. Genet Res 3:139-156.  [MGI Ref ID J:12944]

Liu S; Friel DD. 2008. Impact of the leaner P/Q-type Ca2+ channel mutation on excitatory synaptic transmission in cerebellar Purkinje cells. J Physiol 586(Pt 18):4501-15. [PubMed: 18669535]  [MGI Ref ID J:176384]

McLaren A. 1976. Genetics of the early mouse embryo. Annu Rev Genet 10:361-88. [PubMed: 797312]  [MGI Ref ID J:5768]

Milaire J. 1967. Histochemical observations on the developing foot of normal, oligosyndactylous (Os-plus) and syndactylous (sm-sm) mouse embryos. Arch Biol (Liege) 78(2):223-88. [PubMed: 4305644]  [MGI Ref ID J:5107]

Muhlfeld AS; Spencer MW; Hudkins KL; Kirk E; LeBoeuf RC; Alpers CE. 2004. Hyperlipidemia aggravates renal disease in B6.ROP Os/+ mice. Kidney Int 66(4):1393-402. [PubMed: 15458432]  [MGI Ref ID J:102315]

Naik DV; Valtin H. 1969. Hereditary vasopressin-resistant urinary concentrating defects in mice. Am J Physiol 217(4):1183-90. [PubMed: 5824320]  [MGI Ref ID J:5127]

Ovsepian SV; Friel DD. 2008. The leaner P/Q-type calcium channel mutation renders cerebellar Purkinje neurons hyper-excitable and eliminates Ca2+-Na+ spike bursts. Eur J Neurosci 27(1):93-103. [PubMed: 18093175]  [MGI Ref ID J:132196]

Pravtcheva DD; Wise TL. 2001. Disruption of Apc10/Doc1 in three alleles of oligosyndactylism. Genomics 72(1):78-87. [PubMed: 11247669]  [MGI Ref ID J:81567]

Sorenson CM; Rogers SA; Hammerman MR. 1996. Abnormal renal development in the Os/+ mouse is intrinsic to the kidney. Am J Physiol 271(1 Pt 2):F234-8. [PubMed: 8760267]  [MGI Ref ID J:34503]

Stewart AD; Stewart J. 1969. Studies on syndrome of diabetes insipidus associated with oligosyndactyly in mice. Am J Physiol 217(4):1191-8. [PubMed: 4309975]  [MGI Ref ID J:5128]

Van Valen P. 1966. Oligosyndactylism, an early embryonic lethal in the mouse. J Embryol Exp Morphol 15(2):119-24. [PubMed: 4289631]  [MGI Ref ID J:5017]

Wang Y; Heilig KO; Minto AW; Chen S; Xiang M; Dean DA; Geiger RC; Chang A; Pravtcheva DD; Schlimme M; Deb DK; Wang Y; Heilig CW. 2010. Nephron-deficient Fvb mice develop rapidly progressive renal failure and heavy albuminuria involving excess glomerular GLUT1 and VEGF. Lab Invest 90(1):83-97. [PubMed: 19918242]  [MGI Ref ID J:156400]

Wise TL; Pravtcheva DD. 2004. Oligosyndactylism mice have an inversion of chromosome 8. Genetics 168(4):2099-112. [PubMed: 15611179]  [MGI Ref ID J:95333]

Zalups RK. 1993. The Os/+ mouse: a genetic animal model of reduced renal mass. Am J Physiol 264(1 Pt 2):F53-60. [PubMed: 8430831]  [MGI Ref ID J:3842]

Health & husbandry

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.

Health & Colony Maintenance Information

Animal Health Reports

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

Colony Maintenance

Breeding & HusbandryComments: Os and myd are linked on chromosome 8 and are maintained in repulsion. Os homozygotes are embryonic lethal.

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


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Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $3300.00
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 10 and 14 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* $4290.00
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 10 and 14 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

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   Untyped from the colony
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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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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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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