B6.129S1-Kcne1tm1Sfh/Orl

Status

Available to order

EMMA IDEM:02190
International strain nameB6.129S1-Kcne1tm1Sfh/Orl
Alternative nameIsK-KO
Strain typeTargeted Mutant Strains : Knock-out
Allele/Transgene symbolKcne1tm1Sfh,
Gene/Transgene symbolKcne1

Information from provider

ProviderJacques Barhanin
Provider affiliationIPMC-CNRS
Genetic informationSee ref.: PubMed ID 8982171 (Vetter et al. 1996).
Phenotypic informationDeafness, inner ear defect, shaker behavior.
Breeding historyIsK (Kcne1) +/- backcrossed to C57BL/6J (Charles River) for 11 generations. Current generation >N11F5
References
  • Inner ear defects induced by null mutation of the isk gene.;Vetter D E, Mann J R, Wangemann P, Liu J, McLaughlin K J, Lesage F, Marcus D C, Lazdunski M, Heinemann S F, Barhanin J, ;1996;Neuron;17;1251-64; 8982171
  • The multifaceted phenotype of the knockout mouse for the KCNE1 potassium channel gene.;Warth Richard, Barhanin Jacques, ;2002;American journal of physiology. Regulatory, integrative and comparative physiology;282;R639-48; 11832382
  • Involvement of IsK-associated K+ channel in heart rate control of repolarization in a murine engineered model of Jervell and Lange-Nielsen syndrome.;Drici M D, Arrighi I, Chouabe C, Mann J R, Lazdunski M, Romey G, Barhanin J, ;1998;Circulation research;83;95-102; 9670922

Information from EMMA

Archiving centreInstitut de Transgenose, INTRAGENE, Orléans, France

Disease and phenotype information

MGI allele-associated human disease models

Orphanet associated rare diseases, based on orthologous gene matching

MGI phenotypes (allele matching)
  • abnormal cochlea morphology / MGI
  • abnormal inner ear vestibule morphology / MGI
  • organ of Corti degeneration / MGI
  • absent organ of Corti / MGI
  • abnormal stria vascularis morphology / MGI
  • circling / MGI
  • bidirectional circling / MGI
  • unidirectional circling / MGI
  • hyperactivity / MGI
  • impaired coordination / MGI
  • stereotypic behavior / MGI
  • head bobbing / MGI
  • dehydration / MGI
  • decreased startle reflex / MGI
  • abnormal posture / MGI
  • impaired swimming / MGI
  • impaired righting response / MGI
  • impaired limb coordination / MGI
  • abnormal digestive system physiology / MGI
  • abnormal semicircular canal morphology / MGI
  • increased hematocrit / MGI
  • increased circulating aldosterone level / MGI
  • cochlear ganglion degeneration / MGI
  • increased circulating chloride level / MGI
  • abnormal scala media morphology / MGI
  • prolonged QT interval / MGI
  • abnormal cochlear sensory epithelium morphology / MGI
  • abnormal circulating renin level / MGI
  • abnormal feces composition / MGI
  • abnormal ear physiology / MGI
  • hypokalemia / MGI
  • abnormal crista ampullaris morphology / MGI
  • vestibular hair cell degeneration / MGI
  • vestibular saccular macula degeneration / MGI
  • utricular macular degeneration / MGI
  • cochlear hair cell degeneration / MGI
  • abnormal crista ampullaris neuroepithelium morphology / MGI
  • abnormal vestibular system physiology / MGI
  • abnormal vestibular dark cell morphology / MGI
  • vestibular dark cell degeneration / MGI
  • absent vestibuloocular reflex / MGI
  • head tilt / MGI
  • increased circulating sodium level / MGI
  • collapsed Reissner membrane / MGI
  • absent pinna reflex / MGI
  • absent startle reflex / MGI
  • increased blood osmolality / MGI

Literature references

  • Inner ear defects induced by null mutation of the isk gene.;Vetter D E, Mann J R, Wangemann P, Liu J, McLaughlin K J, Lesage F, Marcus D C, Lazdunski M, Heinemann S F, Barhanin J, ;1996;Neuron;17;1251-64; 8982171
  • The multifaceted phenotype of the knockout mouse for the KCNE1 potassium channel gene.;Warth Richard, Barhanin Jacques, ;2002;American journal of physiology. Regulatory, integrative and comparative physiology;282;R639-48; 11832382
  • Involvement of IsK-associated K+ channel in heart rate control of repolarization in a murine engineered model of Jervell and Lange-Nielsen syndrome.;Drici M D, Arrighi I, Chouabe C, Mann J R, Lazdunski M, Romey G, Barhanin J, ;1998;Circulation research;83;95-102; 9670922

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

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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