B6.129S6-Cln3tm1Nbm/H

Status

Available to order

EMMA IDEM:06145
International strain nameB6.129S6-Cln3tm1Nbm/H
Alternative nameCln3tm1Nbm
Strain typeTargeted Mutant Strains : Knock-out
Allele/Transgene symbolCln3tm1Nbm
Gene/Transgene symbolCln3

Information from provider

ProviderHannah Mitchison
Provider affiliationGenetic Disease Research Branch, National Human Genome Research Institute, National Institutes of Health
Genetic informationCln3 exon 1 (including the start codon) and exons 2-6 replaced with a 1.9-kb HindIII fragment containing a PGK promoter-neomycin resistance-PGK-polyA signal cassette cloned in the reverse orientation.
Phenotypic informationMice maintained on C57BL/6 congenic background over many generations and one rederivation.
Breeding historyThese mice are derived from a congenic C57BL/6 mouse obtained from a collaborator. Rederived (2010), and maintained on C57BL/6 since. Congenic for the last 10 years.
References
  • Targeted disruption of the Cln3 gene provides a mouse model for Batten disease. The Batten Mouse Model Consortium [corrected].;Mitchison H M, Bernard D J, Greene N D, Cooper J D, Junaid M A, Pullarkat R K, de Vos N, Breuning M H, Owens J W, Mobley W C, Gardiner R M, Lake B D, Taschner P E, Nussbaum R L, ;1999;Neurobiology of disease;6;321-34; 10527801
Homozygous fertileyes
Homozygous viableyes
Homozygous matings requiredno
Immunocompromisedno

Information from EMMA

Archiving centreMary Lyon Centre at MRC Harwell, Oxford, United Kingdom
Animals used for archivinghomozygous C57BL/6J
Breeding at archiving centreMales were archived upon arrival. No breeding was performed at the archiving centre.

Disease and phenotype information

MGI allele-associated human disease models

Orphanet associated rare diseases, based on orthologous gene matching

MGI phenotypes (allele matching)
  • abnormal retina morphology / MGI
  • abnormal retinal inner nuclear layer morphology / MGI
  • abnormal retinal ganglion layer morphology / MGI
  • abnormal cerebral cortex morphology / MGI
  • abnormal hippocampus morphology / MGI
  • abnormal brain morphology / MGI
  • abnormal lysosome morphology / MGI
  • abnormal brainstem morphology / MGI
  • abnormal basal ganglion morphology / MGI
  • abnormal dendritic cell physiology / MGI
  • abnormal macrophage physiology / MGI
  • abnormal cell adhesion / MGI
  • abnormal professional antigen presenting cell morphology / MGI
  • decreased macrophage cytokine production / MGI
MGI phenotypes (gene matching)
  • abnormal liver morphology / MGI
  • abnormal hepatocyte morphology / MGI
  • tremors / MGI
  • abnormal cerebral cortex morphology / MGI
  • abnormal hippocampus morphology / MGI
  • abnormal dentate gyrus morphology / MGI
  • abnormal cerebellar Purkinje cell layer / MGI
  • abnormal retina morphology / MGI
  • retinal degeneration / MGI
  • decreased retinal photoreceptor cell number / MGI
  • impaired coordination / MGI
  • abnormal gait / MGI
  • short stride length / MGI
  • polydipsia / MGI
  • limb grasping / MGI
  • polyuria / MGI
  • premature death / MGI
  • abnormal kidney morphology / MGI
  • abnormal brain morphology / MGI
  • abnormal fertility/fecundity / MGI
  • gliosis / MGI
  • abnormal dendritic cell physiology / MGI
  • abnormal macrophage physiology / MGI
  • increased susceptibility to pharmacologically induced seizures / MGI
  • decreased urine osmolality / MGI
  • no phenotypic analysis / MGI
  • abnormal locomotor activation / MGI
  • abnormal cell adhesion / MGI
  • abnormal nervous system morphology / MGI
  • abnormal retinal inner nuclear layer morphology / MGI
  • abnormal postnatal subventricular zone morphology / MGI
  • abnormal lysosome morphology / MGI
  • abnormal retinal ganglion layer morphology / MGI
  • abnormal brainstem morphology / MGI
  • renal/urinary system phenotype / MGI
  • behavior/neurological phenotype / MGI
  • decreased urine potassium level / MGI
  • increased circulating potassium level / MGI
  • abnormal basal ganglion morphology / MGI
  • abnormal retinal neuronal layer morphology / MGI
  • abnormal retinal ganglion cell morphology / MGI
  • abnormal professional antigen presenting cell morphology / MGI
  • increased blood osmolality / MGI
  • abnormal hippocampus pyramidal cell morphology / MGI
  • decreased macrophage cytokine production / MGI

Literature references

  • Targeted disruption of the Cln3 gene provides a mouse model for Batten disease. The Batten Mouse Model Consortium [corrected].;Mitchison H M, Bernard D J, Greene N D, Cooper J D, Junaid M A, Pullarkat R K, de Vos N, Breuning M H, Owens J W, Mobley W C, Gardiner R M, Lake B D, Taschner P E, Nussbaum R L, ;1999;Neurobiology of disease;6;321-34; 10527801

Information on how we integrate external resources can be found here

Order

Availabilities

Requesting frozen sperm or embryos is generally advisable wherever possible, in order to minimise the shipment of live mice.

  • Frozen sperm. Delivered in 4 weeks (after paperwork in place). €1740*
  • Rederivation of mice from frozen stock, delivery time available upon request . €3880*
  • Tissue - Types of tissue, service fee and delivery time available upon request

Due to the dynamic nature of our processes strain availability may change at short notice. The local repository manager will advise you in these circumstances.

* In addition users have to cover all the shipping costs (including the cost for returning dry-shippers, where applicable).

More details on pricing and delivery times

Practical information

Example health report
(Current health report will be provided later)

Material Transfer Agreement (MTA)
MTA will be issued after an order has been submitted.

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Legally binding conditions for the transfer

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