Strain Name:
STOCK Pex2tm1Plf/Mmmh
Stock Number:
032828-MU
Citation ID:
RRID:MMRRC_032828-MU
Other Names:
STOCK Pex2tm1Plf/Mmmh(gene symbol changed from Pxmp3 to Pex2 07/18/12)

Strain Information

Pex2tm1Plf
Name: peroxisomal biogenesis factor 2; targeted mutation 1, Phyllis L Faust
Synonyms: PEX2-
Type: Allele
Species: Mus musculus (mouse)
Chromosome: 3
Alteration at locus: Knockout
Pex2
Name: peroxisomal biogenesis factor 2
Synonyms: PMP35, D3Ertd138e, Pxmp3, Zellweger syndrome homolog
Type: Gene
Species: Mouse
Chromosome: 3
Alteration at locus: Knockout
NCBI: 19302
HGNC: HGNC:9717
Homologene: 269
Genetic Alterations
Exon 5(1.25 kb), which contains the entire coding region for the Pxmp3 gene, is replaced by a neo cassette. The remaining exons and flanking sequence in Pxmp3 gene are not altered.
Genotype Determination
ES Cell Line
R1 derived from (129X1/SvJ x 129S1/Sv)F1-Kitl+
Phenotype
Homozygous: Homozygous mutants in this congenic strain show variable embryonic lethality, starting at ~E11. Approximately 50% of homozygotes survive to birth but are hypotonic, do not feed and die on the day of birth. The brains of these mice have widespread accumulation of neutral lipids in neurons, which begins early in nervous system development.

Heterozygous: Pxmp3tm1Plf heterozygotes are normal and breed normally. Numerous biochemical and morphologic studies have shown no difference between wild-type and Pxmp3tm1Plf+/- mice.
Mammalian Phenotype Terms
Allelic Composition: Pex2tm1Plf/Pex2tm1Plf (Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6 )

Allelic Composition: Pex2tm1Plf/Pex2tm1Plf (Genetic Background: involves: 129S1/Sv * 129X1/SvJ * Swiss Webster )

MeSH Terms
  • Adrenal Glands/pathology
  • Animals
  • Brain/pathology
  • Cell Movement
  • Cerebellum/pathology
  • Cerebral Cortex/pathology
  • Cloning, Molecular
  • Disease Models, Animal
  • Erythrocytes/chemistry
  • Fatty Acids/blood
  • Liver/pathology
  • Membrane Proteins/genetics
  • Mice
  • Mice, Mutant Strains
  • Microbodies/genetics
  • Molecular Sequence Data
  • Morphogenesis/genetics
  • Neurons
  • Peroxisomal Biogenesis Factor 2
  • Plasmalogens/analysis
  • Skull/pathology
  • Zellweger Syndrome/genetics
  • Crosses, Genetic
  • Dendrites/ultrastructure
  • Dietary Fats/pharmacokinetics
  • Docosahexaenoic Acids/metabolism
  • Fatty Acids/metabolism
  • Female
  • Gene Targeting
  • Genes, Lethal
  • Genotype
  • Humans
  • Inclusion Bodies/chemistry
  • Male
  • Membrane Proteins/deficiency
  • Membrane Proteins/metabolism
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Neurons/metabolism
  • Neurons/pathology
  • Organ Specificity
  • Peroxisomes/metabolism
  • Peroxisomes/pathology
  • Plasmalogens/blood
  • Purkinje Cells/pathology
  • Zellweger Syndrome/metabolism
  • Zellweger Syndrome/pathology
  • Brain/embryology
  • Brain/growth & development
  • Brain Chemistry/physiology
  • Cell Differentiation/physiology
  • Cell Movement/physiology
  • Metabolism, Inborn Errors/metabolism
  • Peroxisomal Disorders/genetics
  • Peroxisomal Disorders/metabolism
  • Peroxisomes/physiology
  • Purkinje Cells/physiology
Strain Development
Targeted deletion of the Pxmp3 peroxisome assembly gene was obtained in the R1 embryonic stem cell line. The Pxmp3 ES cells were injected into C57BL/6J blastocysts to generate chimeric mice. Chimeric mice yielding germline transmission were identified. A chimeric mouse was mated with 129S6/SvEvTac mouse and Pxmp3tm1Plf heterozygous pups identified by PCR genotyping. 129S6-Pxmp3tm1Plf+/- mice were mated with wild-type Swiss Webster (SW) mice (from Taconic labs) and Pxmp3tm1Plf heterozygous pups identified by PCR genotyping. SW.129SvJ- Pxmp3tm1Plf mice were backcrossed 12 generations to SW mice.
Suggested Control Mice
  • Wildtype littermates
MMRRC Genetic QC Summary
The MMRRC Centers have developed a genetic QC pipeline using MiniMUGA array genotyping to provide additional information on strain backgrounds for MMRRC congenic and inbred strains. For more information on when data may be available, or to request genotyping for a strain of interest, please contact mmrrc@missouri.edu. Older strains may not have this information.
  • Cell Biology
  • Developmental Biology
  • Internal/Organ
  • Metabolism
  • Models for Human Disease
  • Neurobiology
  • Research Tools
Donor
Phyllis L. Faust, M.D., Ph.D., Columbia University
Primary Reference
  • Faust PL; Hatten ME, Targeted deletion of the PEX2 peroxisome assembly gene in mice provides a model for Zellweger syndrome, a human neuronal migration disorder., J Cell Biol 1997 Dec 1;139(5):1293-305. (Medline PMID: 9382874)
  • Faust PL, Su HM, Moser A, Moser. The peroxisome deficient PEX2 Zellweger mouse: pathologic and biochemical correlates of lipid dysfunction. J Mol Neurosci. 2001 Apr-Jun;16(2-3):289- 97; discussion 317-21. Review. (Medline PMID: 11478384)
  • Faust PL, Banka D, Siriratsivawong R, Ng VG, Wikander TM. Peroxisome biogenesis disorders: the role of peroxisomes and metabolic dysfunction in developing brain. J Inherit Metab Dis. 2005; 28(3):369-83. Review. (Medline PMID: 15868469)

Colony and Husbandry Information

The line is maintained on SW-Pxmp3tm1Plf+/- congenics.

Colony Surveillance Program and Current Health Reports

Mice recovered from a cryo-archive will have health surveillance performed on recipient females. Health reports will be provided prior to shipment. If you require additional health status information, please email mmrrc@missouri.edu.

Order Request Information

Limited quantities of breeder mice (recovered litter) are available from a cryoarchive; recovered litter usually available to ship in 3 to 4 months.

Cryopreserved material may be available upon request, please inquire to mmrrc@missouri.edu for more information.

Distribution of this strain requires submission of the MMRRC Conditions of Use (COU). A link to the COU web form will be provided via email after an order has been placed; the form should be completed then or the email forwarded to your institutional official for completion.

The donor or their institution limits the distribution to non-profit institutions only.

Additional charges may apply for any special requests. Shipping costs are in addition to the basic distribution/resuscitation fees. Information on shipping costs and any additional charges will be provided by the supplying MMRRC facility.

Click button to Request this one strain. (Use the MMRRC Catalog Search to request more than one strain.)
MMRRC Item # Description Distribution Fee / Unit (US $)
*Shipping & Handling not included*
Units Notes
032828-MU-EMBRYO Cryo-preserved embryos $1,038.00 / Non-Profit Aliquot Approximate quantity2 : 20-40 embryos / aliquot
032828-MU-RESUS Litter recovered from cryo-archive $2,028.00 / Non-Profit Litter Recovered litter4; additional fees for any special requests.
Cryopreserved material may be available upon request, please inquire to mmrrc@missouri.edu for more information.

1 The distribution fee covers the expense of rederiving mice from a live mouse; you will receive the resulting litter. The litter will contain at minimum one mutant carrier; the actual number of animals and the gender and genotype ratios will vary. (Typically, multiple breeder pairs can be established from the recovered litter.) Prior to shipment, the MMRRC will provide information about the animals recovered. If you anticipate or find that you need to request specific genotypes, genders or quantities of mice in excess of what is likely from a resuscitated litter, you may discuss available options and pricing with the supplying MMRRC facility.

2 An aliquot contains a sufficient number of embryos (in one or more vials or straws and based on the transfer success rate of the MMRRC facility) to transfer into one to three recipients. The MMRRC makes no guarantee concerning embryo transfer success experienced in the recipient investigator's laboratory. Neither gender nor genotype ratios are guaranteed.

3 An aliquot is one straw or vial with sufficient sperm to recover at least one litter of mice, as per provided protocols, when performed at the MMRRC facility. The MMRRC makes no guarantee concerning the success of these procedures when performed outside the MMRRC facilities.

4 The distribution fee covers the expense of resuscitating mice from the cryo-archive; you will receive the resulting litter. The litter will contain at minimum one mutant carrier; the actual number of animals and the gender and genotype ratios will vary. (Typically, multiple breeder pairs can be established from the recovered litter.) Prior to shipment, the MMRRC will provide information about the animals recovered. If you anticipate or find that you need to request specific genotypes, genders or quantities of mice in excess of what is likely from a resuscitated litter, you may discuss available options and pricing with the supplying MMRRC facility.

To request material from the MMRRC: Please fill out our on-line request form (accessible from the catalog search results page, or click the Request this Strain button in the fees section). If you have questions or need assistance completing this form, you may call Customer Service at (800) 910-2291 (in USA or Canada) or (530) 757-5710 (international calls). Before you call, please have with you: the MMRRC item number, quantity needed, Bill-to and Ship-to contact information.