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Strain Name:

B6C3Fe a/a-Largemyd/J

Stock Number:

000226

Availability:

Repository-Cryopreserved

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General Terms and Conditions

Former Name      B6C3Fe-a/a-Largemyd/+    (Changed: 15-DEC-04 )
Genes & Alleles   Large;   Largemyd;   a;

Product Information

Strain Details

Type JAX® GEMM® Strain - Mutant Stock
Additional information on JAX® GEMM® Strains.
Specieslaboratory mouse
GenerationN19 F1

Strain Development
The myodystrophy mutation, Largemyd, arose spontaneously at the Jackson Laboratory in the lethal spotting strain (LS/LeJ) in 1963. The first affected male was outcrossed to a C57BL/6J female. Matings of a homozygote by heterozygote were carried out as often as possible or as heterozygous pairs. At F43 in 1977 a Largemyd homozygote was mated to a B6C3Fe-a/a hybrid and the strain was maintained by the cross intercross system to the hybrid. When this strain was at N19, it was cryopreserved by mating heterozygous females to heterozygous males to generate embryos.

Related Disease (OMIM) Terms

Facioscapulohumeral Muscular Dystrophy 1A; FSHMD1A
Muscular Dystrophy, Congenital, Type 1d
Mammalian Phenotype Terms assigned by genotype

Largemyd/Large+

        involves: C3HeB/FeJLe * C57BL/6
  • behavior/neurological phenotype
  • limb grasping (MGI Ref ID J:27793)
    • variability with respect to the severity of clasping hind limbs

Largemyd/Largemyd

        involves: C3HeB/FeJLe * C57BL/6
  • muscle phenotype
  • abnormal skeletal muscle fiber morphology (MGI Ref ID J:27793)
  • myopathy (MGI Ref ID J:27793)
    • foci of degeneration typically large and involving 20-50 fibers
  • myositis (MGI Ref ID J:27793)
    • accompanies myocyte necrosis
  • immune system phenotype
  • myositis (MGI Ref ID J:27793)
    • accompanies myocyte necrosis
  • hearing/vestibular/ear phenotype
  • decreased brainstem auditory evoked potential (MGI Ref ID J:27793)
    • prolonged interpeak latencies
    • decreased wave IV amplitude
    • mean wave IV threshold increased

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

Largemyd/Largemyd

        involves: STOCK Edn3ls
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:5670)
    • average life span 17.25 weeks, range 5-39 weeks
  • behavior/neurological phenotype
  • abnormal gait (MGI Ref ID J:5670)
    • hind limbs held close to the body producing a short, shuffling gait
    • hind limbs never extended and dragged
  • limb grasping (MGI Ref ID J:5670)
    • involves hind limbs
    • adduction of hind legs, flexion of the knee, ankles and toes
    • severe contraction of hind limbs sometimes by 3-4 months of age
  • muscle phenotype
  • abnormal muscle morphology (MGI Ref ID J:5670)
    • abnormal skeletal muscle fiber morphology (MGI Ref ID J:5670)
      • variable fiber size
      • loss of striation
      • central migration of nuclei
      • nuclear "rowing"
    • muscle degeneration (MGI Ref ID J:5670)
      • myopathy (MGI Ref ID J:5670)
        • diffuse and progressive myopathy
        • widely distributed focal lesions in skeletal muscles as early as 16 days of age
  • abnormal muscle physiology (MGI Ref ID J:5670)
    • dystrophic muscle (MGI Ref ID J:5670)
    • muscle calcification (MGI Ref ID J:12034)
      • elevated calcium levels in skeletal muscles, particularly the diaphragm
      • heart not affected
    • myositis (MGI Ref ID J:5670)
      • mononuclear cell infiltration of areas surrounding degenerating fibers
  • nervous system phenotype
  • abnormal myelination (MGI Ref ID J:5974)
    • areas where nerves are completely deficient in myelin
  • abnormal spinal nerve morphology (MGI Ref ID J:5974)
    • areas completely deficient in myelin
    • not every root is affected
    • observed in dorsal roots T13 to S1 and Ventral roots L1 to S1 (except L5)
    • L3 and L4 ventral roots most severely affected
  • absent Schwann cells (MGI Ref ID J:5974)
    • usually unmyelinated nerves lack Schwann cells but sometimes Schwann cells present but lacking myelin
  • growth/size phenotype
  • decreased body weight (MGI Ref ID J:5670)
    • organ weights reduced comparably with reduced body weigh
  • postnatal growth retardation (MGI Ref ID J:5670)
    • very severe at weaning
    • growth improved after weaning but mice always small
  • skeleton phenotype
  • abnormal bone structure (MGI Ref ID J:12034)
    • thinning of all bones examined
  • kyphosis (MGI Ref ID J:5670)
    • thoracic kyphosis by 6-8 weeks of age
    • becoming progressively worse with age
  • reproductive system phenotype
  • reduced fertility (MGI Ref ID J:5670)
    • although not sterile, reproduction is very poor
  • immune system phenotype
  • myositis (MGI Ref ID J:5670)
    • mononuclear cell infiltration of areas surrounding degenerating fibers
  • digestive/alimentary phenotype
  • abnormal tongue morphology (MGI Ref ID J:5670)
    • musculature of tongue not affected until later
    • subepithelial fibrosis in tongues of older mice

Largemyd/Largemyd

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

Gene & Allele Details

Allele Symbol Largemyd
Allele Name myodystrophy
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; KIAA0609; MDC1D; 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 a
Allele Name nonagouti

Genotyping Protocols

Largemyd

Related Strains

Strains carrying   Largemyd allele
000300   MYD/Le-Os +/+ Largemyd/J
View Strains carrying   Largemyd     (1 strain)

Strains carrying   a allele
003879   B10;TFLe-a/a T tf/+ tf/J
001538   B6 x B6C3Sn a/A-T(1;9)27H/J
000916   B6 x B6C3Sn a/A-T(5;12)31H/J
000602   B6 x B6C3Sn a/A-T(8;16)17H/J
000618   B6 x FSB/GnEi a/a Ctslfs/J
000577   B6 x STOCK a Oca2p Hps5ru2 Ednrbs/J
000601   B6 x STOCK a/a T(7;18)50H/J
000592   B6 x STOCK T(2;4)13H a/J
000001   B6.C3 A/a Mgrn1md/J
000785   B6;D2-a Es1e/J
000604   B6C3 a/A-T(10;13)199H +/+ Lystbg-J/J or Lystbg-2J/J
002807   B6C3Fe a/a-Meox2fla/J
000224   B6C3Fe a/a-Scyl1mdf/J
001037   B6C3Fe a/a-Agtpbp1pcd/J
000221   B6C3Fe a/a-Alx4lst-J/J
002062   B6C3Fe a/a-Atp7aMo-8J/J
001756   B6C3Fe a/a-Cacng2stg/J
001815   B6C3Fe a/a-Col1a2oim/J
000231   B6C3Fe a/a-Csf1op/J
000209   B6C3Fe a/a-Dh/J
000211   B6C3Fe a/a-Dstdt-J/J
000210   B6C3Fe a/a-Edardl-J/J
000207   B6C3Fe a/a-Edaraddcr/J
000182   B6C3Fe a/a-Eef1a2wst/J
001278   B6C3Fe a/a-Glra1spd/J
000241   B6C3Fe a/a-Glrbspa/J
002875   B6C3Fe a/a-Hoxd13spdh/J
000304   B6C3Fe a/a-Krt71Ca Scn8amed-J/J
000636   B6C3Fe a/a-Lmx1adr-J/J
001280   B6C3Fe a/a-Lse/J
001573   B6C3Fe a/a-MitfMi/J
001035   B6C3Fe a/a-Napahyh/J
000181   B6C3Fe a/a-Otogtwt/J
000278   B6C3Fe a/a-Papss2bm Hps1ep Hps6ru/J
000205   B6C3Fe a/a-Papss2bm/J
002078   B6C3Fe a/a-Pcdh15av-2J/J
000246   B6C3Fe a/a-Pitpnavb/J
001430   B6C3Fe a/a-Ptch1mes/J
000506   B6C3Fe a/a-Qkqk/J
000235   B6C3Fe a/a-Relnrl/J
000237   B6C3Fe a/a-Rorasg/J
000290   B6C3Fe a/a-Sox10Dom/J
000230   B6C3Fe a/a-Tcirg1oc/J
003612   B6C3Fe a/a-Trak1hyrt/J
001512   B6C3Fe a/a-Ttnmdm/J
001607   B6C3Fe a/a-Unc5crcm/J
000005   B6C3Fe a/a-Wc/J
000243   B6C3Fe a/a-Wnt1sw/J
000248   B6C3Fe a/a-Xpl/J
001750   B6C3Fe a/a-XsJ/J
000624   B6C3Fe a/a-anx/J
003020   B6C3Fe a/a-dep/J
002018   B6C3Fe a/a-din/J
002339   B6C3Fe a/a-nma/J
000240   B6C3Fe a/a-soc/J
000063   B6C3Fe a/a-sy/J
001055   B6C3Fe a/a-tip/J
000245   B6C3Fe a/a-tn/J
000296   B6C3Fe-a/a Hoxa13Hd Mcoln3Va-J/J
000019   B6C3Fe-a/a-Itpr1opt/J
001022   B6C3FeF1/J a/a
000971   B6EiC3 a/A-Och/J
000551   B6EiC3 a/A-Tbx15de-H/J
006450   B6EiC3 a/A-Vss/J
000557   B6EiC3-+ a/LnpUl A/J
000503   B6EiC3Sn a/A-Gy/J
001811   B6EiC3Sn a/A-Otcspf-ash/J
002343   B6EiC3Sn a/A-Otcspf/J
000391   B6EiC3Sn a/A-Pax6Sey-Dey/J
001924   B6EiC3Sn a/A-Ts(1716)65Dn
001923   B6EiC3Sn a/A-Ts(417)2Lws Tim/J
000225   C3FeLe.B6 a/a-Ptpn6me/J
000198   C3FeLe.B6-a/J
000291   C3FeLe.Cg-a/a Hm KitlSl Krt71Ca-J/J
001886   C3HeB/FeJLe a/a-gnd/J
000584   C57BL/6J-+ T(1;2)5Ca/a +/J
000284   CWD/LeJ
001057   HPT/LeJ
000260   JGBF/LeJ
000265   MY/HuLeJ
000308   SSL/LeJ
000994   STOCK a Myo5ad Mregdsu/J
000064   STOCK a Tyrp1b Sisi/J
002238   STOCK a Tyrp1b shmy/J
001433   STOCK a skt/J
000579   STOCK a tp/J
000319   STOCK a us/J
002648   STOCK a/a Cln6nclf/J
000317   STOCK a/a Egfrwa2/J
000302   STOCK a/a MitfMi-wh +/+ Itpr1opt/J
000286   STOCK a/a Myo5ad fd/+ +/J
000206   STOCK a/a Tyrc-h/J
001432   STOCK a/a Tyrp1b sks/Tyrp1b +/J
000281   STOCK a/a ma ft/ma ft/J
000312   STOCK stb + a/+ Fignfi a/J
000596   STOCK T(2;11)30H/+ x AEJ-a Gdf5bp-H/J or A/J-a Gdf5bp-J/J
000970   STOCK T(2;16)28H A/T(2;16)28H a/J
000590   STOCK T(2;4)1Sn a/J
000594   STOCK T(2;8)26H a/T(2;8)26H a Tyrp1+/Tyrp1b/J
000623   TR/DiEiJ
View Strains carrying   a     (100 strains)

Strains carrying other alleles of Large
005350   B6.CAST(Cg)-Largevls/Pjn
002491   B6.Cg-Largeenr-Tg(MpbReg)36Pop/J
View Strains carrying other alleles of Large     (2 strains)

Strains carrying other alleles of a
003301   (C57BL/6J x C3H-Eya1bor)F1/J
000251   AEJ.Cg-ae +/a Gdf5bp-H/J
000202   AEJ/Gn-bd/J
000199   AEJ/GnLeJ
000427   B10.CE-H13b Aw/(30NX)SnJ
000420   B10.LP-H13b Aw/Sn
000477   B10.PA-Pldnpa H3e at/SnJ
000419   B10.UW-H3b we Pax1un at/SnJ
000593   B6 x B6CBCa Aw-J/A-Grid2Lc T(2;6)7Ca MitfMi-wh/J
000502   B6 x B6CBCa Aw-J/A-Myo5aflr Gnb5flr/J
000599   B6 x B6CBCa Aw-J/A-T(5;13)264Ca KitW-v/J
002083   B6 x B6EiC3 a/A-T(7;16)235Dn/J
000507   B6 x B6EiC3 a/A-Otcspf/J
002016   B6(Cg)-Aw-J EdaTa-6J Chr YB6-Sxr/EiJ
000552   B6-Aw-J-EdaTa-6J.Cg-Sxr
001730   B6-Aw-J-EdaTa-6J.Cg-Sxrb Hya-/J
000841   B6-Aw-J.CBy-EdaTa-By/J
001809   B6-Aw-J.Cg-EdaTa-6J +/+ ArTfm/J
000600   B6-Gpi1b x B6CBCa Aw-J/A-T(7;15)9H Gpi1a/J
000769   B6.C/(HZ18)By-at-44J/J
000203   B6.C3-Aiy/a/J
000017   B6.C3Fe-Avy/J
000628   B6.CE-A Amy1b Amy2b/J
005505   B6.Cg-Ay Slc7a11sut/LmLlp
000021   B6.Cg-Ay/J
001572   B6.Cg-am-J/J
100409   B6129PF1/J-Aw-J/Aw
004200   B6;CBACa Aw-J/A-Npr2cn-2J/J
000505   B6C3 Aw-J/A-Mutedmu/J
000604   B6C3 a/A-T(10;13)199H +/+ Lystbg-J/J or Lystbg-2J/J
000065   B6C3Fe a/a-we Pax1un at/J
000314   B6CBACa Aw-J/A-EdaTa/J-XO
000501   B6CBACa Aw-J/A-Aifm1Hq/J
001046   B6CBACa Aw-J/A-Grid2Lc/J
000500   B6CBACa Aw-J/A-Gs/J
002703   B6CBACa Aw-J/A-Hydinhy3/J
000247   B6CBACa Aw-J/A-Kcnj6wv/J
000287   B6CBACa Aw-J/A-Plp1jp EdaTa/J
000515   B6CBACa Aw-J/A-SfnEr/J
000242   B6CBACa Aw-J/A-spc/J
000288   B6CBACa Aw-J/A-we a Mafbkr/J
001201   B6CBACaF1/J-Aw-J/A
001752   B6CBCa Aw-J/A-T(7;15)9H/J
006450   B6EiC3 a/A-Vss/J
000557   B6EiC3-+ a/LnpUl A/J
000504   B6EiC3Sn a/A-Cacnb4lh/J
000553   B6EiC3Sn a/A-Egfrwa2 Wnt3avt/J
001811   B6EiC3Sn a/A-Otcspf-ash/J
002343   B6EiC3Sn a/A-Otcspf/J
001923   B6EiC3Sn a/A-Ts(417)2Lws Tim/J
000200   C3FeB6 A/Aw-J-Ankank/J
000638   C3FeB6 A/Aw-J-Spnb4qv-J/J
001203   C3FeB6F1/J A/Aw-J
001272   C3H/HeSnJ-Ahvy/J
000099   C3HeB/FeJ-Avy/J
000338   C57BL/6J Aw-J-EdaTa-6J/J
000258   C57BL/6J-Ai/a/J
000774   C57BL/6J-Asy/a/J
000569   C57BL/6J-Aw-J-EdaTa +/+ ArTfm/J
000051   C57BL/6J-Aw-J/J
000055   C57BL/6J-at-33J/J
000070   C57BL/6J-atd/J
002468   KK.Cg-Ay/J
000262   LS/LeJ
000283   LT.CAST-A/J
001759   STOCK A Tyrc Sha/J
001427   STOCK Aw us/J
View Strains carrying other alleles of a     (67 strains)

Research Applications

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

Largemyd related

Neurobiology Research
Neuromuscular Defects

References

Additional References

Price and Supply Information

Strain Name: B6C3Fe a/a-Largemyd/J
Stock Number: 000226

Price Details

IMPORTANT NOTE: Prices are based on shipping destination. To view prices, select your shipping destination.

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Supply Details

Standard SupplyRepository-Cryopreserved. Must Be Recovered. Please refer to the Supply Notes for further information.
Supply Notes Cryorecovery of Strains Needing Progeny Testing.
The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two untested males and two untested females (two pairs) will be recovered, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the overall recovery time to approximately 25 weeks. However, all pups recovered will be sent.

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 (at least two untested pairs) 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.

Cryorecovery to establish a Dedicated Supply for greater quantities of mice
One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services: Tel: 1-800-422-6423 or 1-207-288-5845; Email: jaxservices@jax.org.
Genomic DNA is available for this strain from the Mouse DNA Resource.

LicensingSee General Terms and Conditions below  

General Terms and Conditions

View JAX® Mice & Services Conditions of Use.

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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