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Strain Name:

B6C3Fe a/a-Dstdt-J/J

Stock Number:

000211

Availability:

Repository-Cryopreserved

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General Terms and Conditions

Former Name      B6C3Fe-a/a-Dstdt-J    (Changed: 15-DEC-04 )
Genes & Alleles   Dst;   Dstdt-J;   a;

Product Information

Strain Details

Type JAX® GEMM® Strain - Mutant Stock
Additional information on JAX® GEMM® Strains.
Specieslaboratory mouse
GenerationN60F1+F1p

Appearance
black
Related Genotype: a/a

Strain Description
Mice homozygous for the dystonia musculorum-J spontaneous mutation (Dstdt-J)are recognizable at 7 to 10 days of age by clasping of the hindlimbs when mice are lifted by the tail. There is increasing incoordination with alternating hyperextension and hyperflexion of the limbs but no obvious paralysis. Many affected animals die before weaning, but some survive several months. The clinical condition becomes relatively stationary after the first phase of deterioration. Histologically, the nervous system shows degenerative changes and progressive loss of nerve fibers in the central and peripheral branches of the sensory ganglion cells of the spinal and cranial nerves, in the central sensory pathways, and in peripheral sensory structures such as skin, Pacinian corpuscles, and muscle spindles. In the PNS, there is some segmental demyelination and other abnormalities of the myelin sheaths.

Strain Development
The dystonia Jackson (dt-J) mutation arose spontaneously in 1957 in the C3HeB/FeDi inbred strain when that strain was at F20. dt-J was initially named athetoid (ah) but was changed to dt-J in 1964 when D. S. Falconer in Edinburgh performed a positive allele test with the original dystonia (dt) mutation (Lane 1958; Falconer 1964). The dt-J allele was maintained heterozygote x heterozygote via test breeding then at F33 was changed to homozygous ovarian transplant backcrossed to C57BL/6J followed by intercross. This reached F33N4 in 1973. At F33N11 the strain was outcrossed once to C3HeB/FeJ A/A, intercrossed once and subsequently maintained by homozygous ovarian transplant crossed to B6C3Fe-a/a F1 then intercross. In 1979 this strain reached N6, in 1983 it reached N18, and in 2004 it reached N59.

Mammalian Phenotype Terms assigned by genotype

Dstdt-J/Dstdt-J

        B6C3Fe a/a-Dstdt-J/J
  • behavior/neurological phenotype
  • abnormal motor coordination/ balance (J:106403)
    • animals exhibit limb flexion when suspended by the tail
    • ataxia (J:106403)
      • mutants are ataxic
    • impaired balance (J:106403)
      • rears and grooming in mutants are usually incomplete due to loss of equilibrium
    • impaired coordination (J:106403)
      • mutants fall more quickly in stationary beam tests than controls; latencies before falling increase over several days
  • abnormal postural reflex (J:106403)
    • mutants showed significant alterations in all measures of postural reflexes other than contact righting
  • abnormal posture (J:106403)
    • animals exhibit folding to the trunk when suspended by the tail
  • abnormal response to tactile stimuli (J:106403)
    • animals are less reactive to touch (smaller escape reflex
  • decreased aggression (J:106403)
    • animals are less reactive to provoked biting
  • decreased startle reflex (J:106403)
    • animals are less reactive to auditory stimuli
  • positive geotaxis (J:106403)
    • animals do not exhibit negative geotaxis and fall off an inclined grid immediately
  • tremors (J:106403)
    • some animals display tremor while at rest and in movement
  • growth/size phenotype
  • decreased body weight (J:106403)
    • mutants have 58% lower mean bodyweights than controls
  • pigmentation phenotype
  • abnormal skin pigmentation (J:106403)
    • animals have paler skin than controls
  • skin/coat/nails phenotype
  • abnormal skin pigmentation (J:106403)
    • animals have paler skin than controls
  • touch/vibrissae phenotype
  • abnormal response to tactile stimuli (J:106403)
    • animals are less reactive to touch (smaller escape reflex
  • vision/eye phenotype
  • abnormal eye distance/ position (J:106403)
    • eyes of majority of mutants are half-closed
    • enophthalmos (J:106403)
      • eyes of mutants seem less prominent
  • hearing/vestibular/ear phenotype
  • decreased startle reflex (J:106403)
    • animals are less reactive to auditory stimuli

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

Dstdt-J/Dstdt-J

        C3HeB/FeJ-Dstdt-J
  • lethality-postnatal
  • postnatal lethality (J:24773)
    • no homozygote has lived beyond 23 days
  • behavior/neurological phenotype
  • abnormal motor coordination/ balance (J:24773)
    • animals make constant swimming motions while on their bellies
    • athetotic walking movements (J:24773)
      • animals are unable to walk normally
  • abnormal posture (J:24773)
    • animals are unable to stand normally

Gene & Allele Details

Allele Symbol Dstdt-J
Allele Name dystonia musculorum Jackson
Common Name(s) ah; athetoid; dt;
Strain of OriginC3HeB
Gene Symbol and Name Dst, dystonin
Chromosome 1
Gene Common Name(s) 2310001O04Rik; A830042E19Rik; AW554249; BP240; BPA; BPAG1; BPAG1-n; Bpag; Bpag1; CATX-15; D6S1101; DKFZp564B2416; DMH; DT; FLJ46791; KIAA0465; KIAA1470; MACF2; RIKEN cDNA 2310001O04 gene; RIKEN cDNA A830042E19 gene; ah; athetoid; bullous pemphigoid antigen; bullous pemphigoid antigen 1; bullous pemphigoid antigen 1, 230 kDa; dt; dystonia musculorum; expressed sequence AW554249; mKIAA0728; neuroscience mutagenesis facility, 203; neuroscience mutagenesis facility, 339; nmf203; nmf339;
General Note At least 27 remutations have occurred at The Jackson Laboratory. The first, Dstdt-J, occurred spontaneously in C3HeB, and was originally designated athetoid (J:24773). It was soon shown to be an allele of the dt mutation, however (J:160)
 
Allele Symbol a
Allele Name nonagouti

Control Information

  Allele   Control
 Dstdt-J  Untyped from the colony
 
  Considerations for Choosing Controls

Related Strains

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
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
000226   B6C3Fe a/a-Largemyd/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 Dst
005633   B6;CByJ-Dstdt-38J/J
004829   C57BL/6J-Dstdt-36J/J
005560   C57BL/6J-Dstdt-37J/J
View Strains carrying other alleles of Dst     (3 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:

Dstdt-J related

Developmental Biology Research
Neurodevelopmental Defects

Mouse/Human Gene Homologs
bullous pemphigoid

Neurobiology Research
Myelination Defects
Neurodevelopmental Defects
Neuromuscular Defects

References

Additional References

Price and Supply Information

Strain Name: B6C3Fe a/a-Dstdt-J/J
Stock Number: 000211

Price Details

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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.
This strain is included in the Mouse Mutant Resource collection.
Genomic DNA is available for this strain from the Mouse DNA Resource.

LicensingSee General Terms and Conditions below  
Control InformationView Control Information in Strain Details.

General Terms and Conditions

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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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