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Strain Name:

B6;129-Plp1tm1Kan/J

Stock Number:

003255

Availability:

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General Terms and Conditions

Former Name      B6;129-Plptm1Kan/J    (Changed: 05-JAN-05 )
Genes & Alleles   Plp1;   Plp1tm1Kan;

Product Information

Strain Details

Type JAX® GEMM® Strain - Mutant Stock
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Targeted Mutation
Specieslaboratory mouse
Donating Investigator Klaus-Armin Nave,   Max-Planck-Institute of Experimental Med
GenerationF?+2

Strain Description
The gene for myelin proteolipid protein (Plp) lies on the X chromosome. Mice homozygous or hemizygous for the Plptm1Kan targeted mutation are viable and fertile. Although they lack expression of the PLP protein, they show no sign of motor deficits, tremors or seizures, in contrast to most naturally occurring PLP mutants. PLP is a major membrane component of CNS myelin, and many PLP mutants show defects in myelin sheath formation. However, Plptm1Kan mice do not exhibit this dysmyelination. Some ultrastructural differences are observed in the myelin of Plptm1Kan mutant mice when compared to controls, including a less distinct difference between the major dense line (MDL) and the intraperiod line (IPL), which corresponds to reduced physical stability of the myelin sheath and suggests a function of PLP in maintenance of myelin architecture. Plptm1Kan mutant mice show no evidence of myelin breakdown with age.

Mammalian Phenotype Terms assigned by genotype

Plp1tm1Kan/Y

        involves: 129S1/Sv * 129X1/SvJ * C57BL/6J
  • nervous system phenotype
  • abnormal axon morphology (MGI Ref ID J:38856)
    • in mice over 2 months of age, some axonal spheroids
    • abnormal myelin sheath morphology (MGI Ref ID J:38856)
      • myelin is compacted but the distinction between major dense lines and intraperiod lines is less than in wild type; however, the proportion of myelinated axons, myelin sheath thickness and size range of myelinated fibers are similar to wild type
      • in a few areas double intraperiod lines are seen and myelin sheaths are more susceptible to damage during the fixation process compared to wild type
  • behavior/neurological phenotype
  • *normal* behavior/neurological phenotype (MGI Ref ID J:38856)
    • no signs of motor dysfunction or impaired coordination are detected

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

Plp1tm1Kan/Plp1+

        involves: 129S1/Sv * 129X1/SvJ
  • nervous system phenotype
  • abnormal axon morphology (MGI Ref ID J:48031)
    • axonal swelling and degeneration are seen but less frequently than in hemizygous males; however all optic nerve axons have normal myelin sheaths

Plp1tm1Kan/Y

        involves: 129S1/Sv * 129X1/SvJ
  • nervous system phenotype
  • abnormal axon morphology (MGI Ref ID J:48031)
    • at 6 - 8 weeks, focal axonal swellings containing organelles are seen mostly in areas where small diameter axons predominate throughout the white and gray matter
    • by 1 year, numerous large axonal swellings are seen in the optic nerve and spinal cord with some also seen in Purkinje cell axons
    • after 1 year, axonal degeneration is seen in the optic nerve and fasciculus gracilis; however, no signs of peripheral neuropathy are seen
    • abnormal myelin sheath morphology (MGI Ref ID J:48031)
      • in areas of axonal swelling the myelin sheath becomes attenuated and is eventually lost though slippage
  • abnormal microglial cell morphology (MGI Ref ID J:48031)
    • increased numbers of microglia accompany degenerative changes
  • abnormal oligodendrocyte morphology (MGI Ref ID J:48031)
    • occasionally inner tongue processes of pligdendrocytes contain degenerated organelles
    • primary glial cultures have increased numbers of oligodendrocytes starting at the second division and persisting through at least the fourteenth division; however no increase in oligodendrocyte proliferation is seen
    • in primary glial cultures oligodendrocyte sheets appear larger, vacuolated, and broken suggesting decreased stability of the myelin sheet
  • astrocytosis (MGI Ref ID J:48031)
    • mild astrocytosis accompanies degenerative changes
  • demyelination (MGI Ref ID J:48031)
    • evidence of demyelination is seen at 22 months of age
  • behavior/neurological phenotype
  • abnormal locomotor coordination (MGI Ref ID J:48031)
    • at 16 months of age impaired performance in a rotarod test is seen
    • abnormal gait (MGI Ref ID J:48031)
      • older mice develop a slow gait
  • impaired coordination (MGI Ref ID J:48031)
    • at 16 months of age impaired performance in a rotarod test is seen
  • hematopoietic system phenotype
  • abnormal microglial cell morphology (MGI Ref ID J:48031)
    • increased numbers of microglia accompany degenerative changes
  • immune system phenotype
  • abnormal microglial cell morphology (MGI Ref ID J:48031)
    • increased numbers of microglia accompany degenerative changes

Gene & Allele Details

Allele Symbol Plp1tm1Kan
Allele Name targeted mutation 1, Klaus-Armin Nave
Common Name(s) PLP/DM20 null; Plp-;
Mutation Made By Klaus-Armin Nave,   Max-Planck-Institute of Experimental Med
Strain of Origin(129X1/SvJ x 129S1/Sv)F1-Kitl+
ES Cell Line NameR1
ES Cell Line Strain(129X1/SvJ x 129S1/Sv)F1-Kitl<+>
Gene Symbol and Name Plp1, proteolipid protein (myelin) 1
Chromosome X
Gene Common Name(s) DM20; MMPL; PLP; PLP/DM20; PMD; Plp; SPG2; jimpy; jp; msd; myelin synthesis deficiency; proteolipid protein (myelin); rsh; rump shaker;
Molecular Note A neomycin resistance cassette introduced by homologous recombination replaced the 3' part of exon 1,including the translation start codon and a 2.5 kb fragment of the first intron of Plp ("PLP/DM20 gene"). Western blot analysis showed absence of both PLP and DM20 proteolipids proteins in hemizygous mutant mice. [MGI Ref ID J:38856]

Related Strains

Strains carrying other alleles of Plp1
005975   B6.Cg-Tg(Plp1-cre/ESR1)3.16Pop/J
000287   B6CBACa Aw-J/A-Plp1jp EdaTa/J
View Strains carrying other alleles of Plp1     (2 strains)

Research Applications

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

Plp1tm1Kan related

Cancer Research
Genes Regulating Growth and Proliferation

Cell Biology Research
Genes Regulating Growth and Proliferation

Developmental Biology Research
Embryonic Lethality (Homozygous)
Neurodevelopmental Defects
Postnatal Mortality (Homozygous)

Neurobiology Research
Myelination Defects
Neurodevelopmental Defects
Neurotrophic Factor Defects

References

Selected Reference(s)

Klugmann M; Schwab MH; Puhlhofer A; Schneider A; Zimmermann F ; Griffiths IR ; Nave KA. 1997. Assembly of CNS myelin in the absence of proteolipid protein. Neuron 18(1):59-70. [PubMed: 9010205]  [MGI Ref ID J:38856]

Additional References

Price and Supply Information

Strain Name: B6;129-Plp1tm1Kan/J
Stock Number: 003255

Price Details

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Supply Details

Standard SupplyRepository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information.
Supply Notes Cryorecovery - Standard.
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 males and two females (two pairs) will be provided, 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 delivery time to approximately 25 weeks. At least one member of each pair will be of known genotype and will carry the mutation if it is a mutant strain. Please note that 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. Price represents a repository maintenance fee, which includes the cost of recovery of the strain from the cryopreservation resource and the periodic replacement of the frozen embryos used for recovery.

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 Induced Mutant Resource Colony collection.

LicensingSee General Terms and Conditions below  

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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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