B6.129S6-Cln3^tm1Nbm/H

Status	Available to order
EMMA ID	EM:06145
Citation information	RRID:IMSR_EM:06145 Research Resource Identifiers (RRID) are persistent unique ID numbers assigned to help researchers cite key resources (e.g. antibodies, model organisms and software projects) in the biomedical literature to improve transparency and reproducibility in research. See https://www.rrids.org/ for more information.
International strain name	B6.129S6-Cln3^tm1Nbm/H
Alternative name	Cln3tm1Nbm
Strain type	Targeted Mutant Strains : Knock-out
Allele/Transgene symbol	Cln3^tm1Nbm
Gene/Transgene symbol	Cln3

Information from provider

Provider	Hannah Mitchison
Provider affiliation	Genetic Disease Research Branch, National Human Genome Research Institute, National Institutes of Health
Genetic information	Cln3 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 information	Mice maintained on C57BL/6 congenic background over many generations and one rederivation.
Breeding history	These 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 fertile	yes
Homozygous viable	yes
Homozygous matings required	no
Immunocompromised	no

Information from EMMA

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

Disease and phenotype information

MGI allele-associated human disease models

neuronal ceroid lipofuscinosis 3 / DOID:0110731

Orphanet associated rare diseases, based on orthologous gene matching

CLN3 disease / Orphanet_228346

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

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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).

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Practical information

Example health report
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Material Transfer Agreement (MTA)
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Legally binding conditions for the transfer

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B6.129S6-Cln3tm1Nbm/H