C3H.B6J-Col4a4m1HPde6b+/H

Status

Available to order

EMMA IDEM:15937
Citation informationRRID:IMSR_EM:15937 

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 nameC3H.B6J-Col4a4m1HPde6b+/H
Alternative nameC3H.B6J-Col4a4Pde6b<+>/H
Strain typeInduced Mutant Strains
Allele/Transgene symbolCol4a1m1H
Gene/Transgene symbolCol4a1

Information from provider

Provider MRC, Medical Research Council
Provider affiliationMary Lyon Centre at MRC Harwell
Genetic informationOriginal mutant (VIS/66.2b) was offspring of "sighted" C3H female (i.e. the congenic C3H stock carrying the BALB/c version of the Pde6b gene) mated to C57BL/6J male that received ENU. The causative mutation is a novel stop codon in Col4a4. This is an ENU model of Alport disease.
Phenotypic informationThese mice model autosomal recessive Alport syndrome and exhibit extensive glomerulonephropathy with increased levels of serum urea and creatinine. Homs exhibit weight loss, and thinner appearance. Regular weights required to monitor homs.
Breeding historyOriginal mutant (VIS/66.2b) was offspring of "sighted" C3H female (i.e. the congenic C3H stock carrying the BALB/c version of the Pde6b gene) mated to C57BL/6J male that received ENU. Now congenic on sighted C3H.
References
  • Modification of an aggressive model of Alport Syndrome reveals early differences in disease pathogenesis due to genetic background.;Falcone Sara, Wisby Laura, Nicol Thomas, Blease Andrew, Starbuck Becky, Parker Andrew, Sanderson Jeremy, Brown Steve D M, Scudamore Cheryl L, Pusey Charles D, Tam Frederick W K, Potter Paul K, ;2019;Scientific reports;9;20398; 31892712
Homozygous fertilenot known
Homozygous viableno
Homozygous matings requirednot known
Immunocompromisednot known

Information from EMMA

Archiving centreMary Lyon Centre at MRC Harwell, Oxford, United Kingdom

Disease and phenotype information

MGI phenotypes (gene matching)
  • decreased hematocrit / MGI
  • delayed kidney development / MGI
  • weakness / MGI
  • myopathy / MGI
  • dystrophic muscle / MGI
  • abnormal cerebral cortex morphology / MGI
  • delaminated cerebral cortex / MGI
  • abnormal hippocampus morphology / MGI
  • decreased body weight / MGI
  • decreased body size / MGI
  • microphthalmia / MGI
  • abnormal lens morphology / MGI
  • cataract / MGI
  • abnormal cornea morphology / MGI
  • corneal opacity / MGI
  • irregularly shaped pupil / MGI
  • abnormal iris morphology / MGI
  • abnormal retina morphology / MGI
  • anemia / MGI
  • abnormal blood vessel morphology / MGI
  • polyuria / MGI
  • kidney inflammation / MGI
  • intracranial hemorrhage / MGI
  • intracerebral hemorrhage / MGI
  • reduced fertility / MGI
  • infertility / MGI
  • respiratory distress / MGI
  • seizures / MGI
  • premature death / MGI
  • abnormal eye morphology / MGI
  • abnormal kidney morphology / MGI
  • abnormal kidney physiology / MGI
  • abnormal brain morphology / MGI
  • abnormal retinal vasculature morphology / MGI
  • abnormal renal glomerular capsule morphology / MGI
  • albuminuria / MGI
  • decreased hemoglobin content / MGI
  • decreased erythrocyte cell number / MGI
  • increased urine microalbumin level / MGI
  • decreased urine osmolality / MGI
  • abnormal lens capsule morphology / MGI
  • astrocytosis / MGI
  • vitreal fibroplasia / MGI
  • nervous system phenotype / MGI
  • abnormal Reichert's membrane morphology / MGI
  • abnormal kidney papilla morphology / MGI
  • abnormal basement membrane morphology / MGI
  • abnormal kidney collecting duct morphology / MGI
  • abnormal proximal convoluted tubule morphology / MGI
  • dilated kidney collecting duct / MGI
  • hematuria / MGI
  • abnormal renal glomerulus morphology / MGI
  • abnormal podocyte morphology / MGI
  • corneal vascularization / MGI
  • increased blood urea nitrogen level / MGI
  • brainstem hemorrhage / MGI
  • abnormal retinal neuronal layer morphology / MGI
  • eye hemorrhage / MGI
  • optic nerve hypoplasia / MGI
  • abnormal retinal ganglion cell morphology / MGI
  • abnormal podocyte foot process morphology / MGI
  • abnormal podocyte slit diaphragm morphology / MGI
  • podocyte foot process effacement / MGI
  • abnormal brain pia mater morphology / MGI
  • dilated proximal convoluted tubules / MGI
  • buphthalmos / MGI
  • bruising / MGI
  • decreased grip strength / MGI
  • increased circulating creatine kinase level / MGI
  • abnormal retinal blood vessel pattern / MGI
  • abnormal retina inner limiting membrane morphology / MGI
  • anterior subcapsular cataracts / MGI
  • anterior polar cataracts / MGI
  • sutural cataracts / MGI
  • total cataracts / MGI
  • postnatal lethality, incomplete penetrance / MGI
  • perinatal lethality, incomplete penetrance / MGI
  • prenatal lethality, complete penetrance / MGI
  • embryonic lethality, complete penetrance / MGI
  • lethality throughout fetal growth and development, complete penetrance / MGI
  • prenatal lethality, incomplete penetrance / MGI
  • abnormal parietal endoderm morphology / MGI
  • abnormal glomerular mesangium morphology / MGI
  • abnormal renal glomerulus basement membrane morphology / MGI
  • enlarged brain ventricles / MGI
  • renal cast / MGI
  • erythrocyturia / MGI
  • anterior iris synechia / MGI
  • abnormal glomerular capsule parietal layer morphology / MGI
  • increased glomerular capsule space / MGI
  • parietal capsular epithelium metaplasia / MGI
  • dilated glomerular capillary / MGI
  • renal glomerulus cysts / MGI
  • vacuolated lens / MGI
  • abnormal kidney collecting duct epithelium morphology / MGI
  • abnormal urine albumin level / MGI
  • decreased collagen level / MGI

Literature references

  • Modification of an aggressive model of Alport Syndrome reveals early differences in disease pathogenesis due to genetic background.;Falcone Sara, Wisby Laura, Nicol Thomas, Blease Andrew, Starbuck Becky, Parker Andrew, Sanderson Jeremy, Brown Steve D M, Scudamore Cheryl L, Pusey Charles D, Tam Frederick W K, Potter Paul K, ;2019;Scientific reports;9;20398; 31892712

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

Genotyping protocol

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

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

EMMA conditions
Legally binding conditions for the transfer

Right strain for your research?

The information provided on this page is, to the best of EMMA’s knowledge, based on data supplied by the original provider. End users are responsible for reviewing these details and for validating the strain and its suitability for their experimental use.​
Not found what you were looking for? Search here for other strains available from EMMA.


Search
INFRAFRONTIER® and European Mouse Mutant Archive - EMMA® are registered trademarks at the European Union Intellectual Property Office (EUIPO).