STOCK Selenontm1.2Mred/Orl

Status

Available to order

EMMA IDEM:02138
International strain nameSTOCK Selenontm1.2Mred/Orl
Alternative nameSEPN1 -/- (K51-326 L-)
Strain typeTargeted Mutant Strains : Knock-out
Allele/Transgene symbolSelenontm1.2Mred
Gene/Transgene symbolSelenon

Information from provider

ProviderAlain Lescure
Provider affiliationCNRS - Universite Louis Pasteur
Genetic informationDeletion of the third exon by cre-induced recombination leading to a frameshift and premature stop of translation in exon 6.
Phenotypic informationUnder investigation. No direct muscular defects observed, but possibly induced by sub-optimal vitamin E supply.
Breeding history1 backcross to C57BL/6J.
References
  • Increased muscle stress-sensitivity induced by selenoprotein N inactivation in mouse: a mammalian model for SEPN1-related myopathy.;Rederstorff Mathieu, Castets Perrine, Arbogast Sandrine, Lainé Jeanne, Vassilopoulos Stéphane, Beuvin Maud, Dubourg Odile, Vignaud Alban, Ferry Arnaud, Krol Alain, Allamand Valérie, Guicheney Pascale, Ferreiro Ana, Lescure Alain, ;2011;PloS one;6;e23094; 21858002
  • Satellite cell loss and impaired muscle regeneration in selenoprotein N deficiency.;Castets Perrine, Bertrand Anne T, Beuvin Maud, Ferry Arnaud, Le Grand Fabien, Castets Marie, Chazot Guillaume, Rederstorff Mathieu, Krol Alain, Lescure Alain, Romero Norma B, Guicheney Pascale, Allamand Valérie, ;2011;Human molecular genetics;20;694-704; 21131290
  • Selenoprotein N is dynamically expressed during mouse development and detected early in muscle precursors.;Castets Perrine, Maugenre Svetlana, Gartioux Corine, Rederstorff Mathieu, Krol Alain, Lescure Alain, Tajbakhsh Shahragim, Allamand Valérie, Guicheney Pascale, ;2009;BMC developmental biology;9;46; 19698141

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

IMPC phenotypes (gene matching)
  • hydrocephalus / IMPC
  • process of degenerative change / IMPC
  • vertebral fusion / IMPC
MGI phenotypes (allele matching)
  • abnormal muscle regeneration / MGI
  • abnormal muscle physiology / MGI
  • decreased satellite cell number / MGI
  • skeletal muscle atrophy / MGI
  • kyphosis / MGI
  • abnormal skeletal muscle morphology / MGI
  • decreased skeletal muscle mass / MGI
  • decreased skeletal muscle fiber size / MGI
  • decreased skeletal muscle fiber diameter / MGI
  • centrally nucleated skeletal muscle fibers / MGI
  • abnormal skeletal muscle fiber type ratio / MGI
  • decreased gastrocnemius weight / MGI
  • decreased extensor digitorum longus weight / MGI
  • decreased soleus weight / MGI
  • decreased tibialis anterior weight / MGI
  • decreased susceptibility to weight gain / MGI
  • decreased quadriceps weight / MGI
MGI phenotypes (gene matching)
  • kyphosis / MGI
  • abnormal muscle regeneration / MGI
  • abnormal skeletal muscle morphology / MGI
  • abnormal lung development / MGI
  • hypoactivity / MGI
  • abnormal lung volume / MGI
  • abnormal muscle physiology / MGI
  • abnormal respiratory system physiology / MGI
  • decreased vertical activity / MGI
  • decreased satellite cell number / MGI
  • vertebral fusion / MGI
  • decreased skeletal muscle mass / MGI
  • increased apoptosis / MGI
  • decreased skeletal muscle fiber size / MGI
  • decreased skeletal muscle fiber diameter / MGI
  • centrally nucleated skeletal muscle fibers / MGI
  • abnormal skeletal muscle fiber type ratio / MGI
  • skeletal muscle atrophy / MGI
  • decreased gastrocnemius weight / MGI
  • decreased extensor digitorum longus weight / MGI
  • decreased soleus weight / MGI
  • decreased tibialis anterior weight / MGI
  • skeletal muscle hypertrophy / MGI
  • decreased susceptibility to weight gain / MGI
  • decreased quadriceps weight / MGI
  • increased lung compliance / MGI
  • abnormal pulmonary alveolar sac morphology / MGI
  • decreased lung elastance / MGI
  • enhanced exercise endurance / MGI

Literature references

  • Increased muscle stress-sensitivity induced by selenoprotein N inactivation in mouse: a mammalian model for SEPN1-related myopathy.;Rederstorff Mathieu, Castets Perrine, Arbogast Sandrine, Lainé Jeanne, Vassilopoulos Stéphane, Beuvin Maud, Dubourg Odile, Vignaud Alban, Ferry Arnaud, Krol Alain, Allamand Valérie, Guicheney Pascale, Ferreiro Ana, Lescure Alain, ;2011;PloS one;6;e23094; 21858002
  • Satellite cell loss and impaired muscle regeneration in selenoprotein N deficiency.;Castets Perrine, Bertrand Anne T, Beuvin Maud, Ferry Arnaud, Le Grand Fabien, Castets Marie, Chazot Guillaume, Rederstorff Mathieu, Krol Alain, Lescure Alain, Romero Norma B, Guicheney Pascale, Allamand Valérie, ;2011;Human molecular genetics;20;694-704; 21131290
  • Selenoprotein N is dynamically expressed during mouse development and detected early in muscle precursors.;Castets Perrine, Maugenre Svetlana, Gartioux Corine, Rederstorff Mathieu, Krol Alain, Lescure Alain, Tajbakhsh Shahragim, Allamand Valérie, Guicheney Pascale, ;2009;BMC developmental biology;9;46; 19698141

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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*

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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