B6.129S4-Gjb1tm1Kwi/Cnrm

Status

Available to order

EMMA IDEM:00243
International strain nameB6.129S4-Gjb1tm1Kwi/Cnrm
Alternative nameCx32 KO
Strain typeTargeted Mutant Strains : Knock-out
Allele/Transgene symbolGjb1tm1Kwi,
Gene/Transgene symbolGjb1

Information from provider

ProviderKlaus WILLECKE
Provider affiliationMolekulargenetik, Institut fuer Genetik, Universitaet Bonn
Genetic informationExon 2 including the whole open reading frame of gap junction membrane channel protein beta 1 (Gjb1; connexin 32) is homologously replaced by a selection marker gene (neomycin resistance) which is still in the genome.
Phenotypic informationDegeneration of myelin in older mice.
Breeding historyAfter blastocyst injection the chimeras were bred with C57BL/6NCrl to obtain brown coat coloured offspring. This brown offspring was backcrossed to C57BL/6NCrl more than three times.
References
  • Defective propagation of signals generated by sympathetic nerve stimulation in the liver of connexin32-deficient mice.;Nelles E, Bützler C, Jung D, Temme A, Gabriel H D, Dahl U, Traub O, Stümpel F, Jungermann K, Zielasek J, Toyka K V, Dermietzel R, Willecke K, ;1996;Proceedings of the National Academy of Sciences of the United States of America;93;9565-70; 8790370

Information from EMMA

Archiving centreCNR, Consiglio Nazionale delle Ricerche, Monterotondo, Italy
Breeding at archiving centreBackcrossed to C57BL/6J

Disease and phenotype information

MGI allele-associated human disease models

Orphanet associated rare diseases, based on orthologous gene matching

MGI phenotypes (allele matching)
  • demyelination / MGI
  • abnormal Schwann cell morphology / MGI
  • abnormal nervous system morphology / MGI
  • abnormal myelin sheath morphology / MGI
  • abnormal myelination / MGI
  • behavior/neurological phenotype / MGI
  • abnormal nerve conduction / MGI
  • abnormal axon morphology / MGI
  • abnormal liver morphology / MGI
  • decreased body weight / MGI
  • nervous system phenotype / MGI

Literature references

  • Defective propagation of signals generated by sympathetic nerve stimulation in the liver of connexin32-deficient mice.;Nelles E, Bützler C, Jung D, Temme A, Gabriel H D, Dahl U, Traub O, Stümpel F, Jungermann K, Zielasek J, Toyka K V, Dermietzel R, Willecke K, ;1996;Proceedings of the National Academy of Sciences of the United States of America;93;9565-70; 8790370
  • Connexin32 mutations cause loss of function in Schwann cells and oligodendrocytes leading to PNS and CNS myelination defects.;Sargiannidou Irene, Vavlitou Natalie, Aristodemou Sophia, Hadjisavvas Andreas, Kyriacou Kyriacos, Scherer Steven S, Kleopa Kleopas A, ;2009;The Journal of neuroscience : the official journal of the Society for Neuroscience;29;4736-49; 19369543
  • Disruption of oligodendrocyte gap junctions in experimental autoimmune encephalomyelitis.;Markoullis Kyriaki, Sargiannidou Irene, Gardner Christopher, Hadjisavvas Andreas, Reynolds Richard, Kleopa Kleopas A, ;2012;Glia;60;1053-66; 22461072
  • Transgenic replacement of Cx32 in gap junction-deficient oligodendrocytes rescues the phenotype of a hypomyelinating leukodystrophy model.;Schiza Natasa, Sargiannidou Irene, Kagiava Alexia, Karaiskos Christos, Nearchou Marianna, Kleopa Kleopas A, ;2015;Human molecular genetics;24;2049-64; 25524707

Order

Availabilities

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

  • Frozen embryos. Delivered in 4 weeks (after paperwork in place). €1740*
  • Rederivation of mice from frozen stock, delivery time available upon request . €3880*

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)
For this strain no provider MTA is needed. Distribution is based on the EMMA conditions only.

EMMA conditions
Legally binding conditions for the transfer

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