Strain Data Sheet

RBRC03654

Information1

Image
BRC No.RBRC03654
TypeTargeted MutationCartagena
SpeciesMus musculus
Strain nameB6;129-Arx<tm(GCG)Kki>/7-1Rbrc
Former Common nameArx (GCG)7-1 KI (B6)
H-2 Haplotype
ES Cell lineCCE/EK.CCE [129S/SvEv-Gpi1<c>]
Background strain
Appearance
Strain developmentDeveloped by Kunio Kitamura, Mitsubishi Kagaku Institute of Life Sciences. CCE/EK.CCE ES cells were used to generate the mutant mice.
Strain descriptionArx GCG 7 repeats knock-in mice. 7 repeats of the GCG triple were inserted at the codon 330 of Arx gene. C57BL/6 mixed background (RBRC03654 and RBRC03656), 129 background (RBRC03655), C3H mixed background (RBRC03657), ICR mixed background (RBRC03665).
Colony maintenanceHeterozygote x Wild-type [C57BL/6JJcl]
ReferencesHum. Mol. Genet., 18, 3708-3724 (2009). 19605412

Health Report

Examination Date / Room / Racknull

Gene

Gene info
Gene symbolGene nameChr.Allele symbolAllele nameCommon namesPromoter
Arxaristaless related homeoboxXArx<tm4Kki>targeted mutation 4, Kunio Kitamura

Gene symbolGene nameChr.Allele symbolAllele nameCommon namesPromoter
neoneomycin resistance gene (E. coli)Xherpes simplex virus thymidine kinase promoter (HSV tk promoter)

Gene symbolGene nameChr.Allele symbolAllele nameCommon namesPromoter
SV40 polyA signalX

Information2

Donor DNAherpes simplex virus thymidine kinase promoter (HSV tk promoter), E. coli Neomycin resistance gene, SV40 polyA signal, mouse ARX genomic DNA
Research applicationMouse Models for Human Disease
Neurobiology Research
Specific Term and ConditionsThe BIOLOGICAL RESOURCE shall be used only for academic research purposes by a non-profit organization.
The RECIPIENT of a non-profit organization must prior contact RIKEN BRC and negotiate with Mitsubishi Chemical Corporation to use the BIOLOGICAL RESOURCE for purposes of other than academic research.
In publishing the research results to be obtained by use of the BIOLOGICAL RESOURCE in relevant journals and meetings, the RECIPIENT must stipulate the source of the BIOLOGICAL RESOURCE and an acknowledgment to Mitsubishi Chemical Corporation.
The RECIPIENT agrees that RIKEN BRC informs annually to Mitsubishi Chemical Corporation of RECIPIENT organization, RECIPIENT name, the specific research purpose and the date of distribution.
DepositorKunio Kitamura (Mitsubishi Kasei Institute of Life Sciences)
Strain Statusan icon for Frozen embryosFrozen embryos
Strain AvailabilityRecovered litters from cryopreserved embryos (2 to 4 months)
Cryopreserved embryos (within 1 month)
Additional Info.Necessary documents for ordering:
  1. Order form (Japanese / English)
  2. Category I MTA: MTA for distribution with RIKEN BRC (Japanese / English)
  3. Acceptance of responsibility for living modified organism (Japanese / English)

Genotyping protocol -PCR-

BRC mice in Publications

Beguin S, Crépel V, Aniksztejn L, Becq H, Pelosi B, Pallesi-Pocachard E, Bouamrane L, Pasqualetti M, Kitamura K, Cardoso C, Represa A.
An epilepsy-related ARX polyalanine expansion modifies glutamatergic neurons excitability and morphology without affecting GABAergic neurons development.
Cereb Cortex 23(6) 1484-94(2013) 22628459

Drongitis D, Caterino M, Verrillo L, Santonicola P, Costanzo M, Poeta L, Attianese B, Barra A, Terrone G, Lioi MB, Paladino S, Di Schiavi E, Costa V, Ruoppolo M, Miano MG.
Deregulation of microtubule organization and RNA metabolism in Arx models for Lissencephaly and developmental epileptic encephalopathy.
Hum Mol Genet (2022) 35094084

Lee K, Mattiske T, Kitamura K, Gecz J, Shoubridge C.
Reduced polyalanine-expanded Arx mutant protein in developing mouse subpallium alters Lmo1 transcriptional regulation.
Hum Mol Genet 23(4) 1084-94(2014) 24122442

Loring KE, Mattiske T, Lee K, Zysk A, Jackson MR, Noebels JL, Shoubridge C.
Early 17β-estradiol treatment reduces seizures but not abnormal behaviour in mice with expanded polyalanine tracts in the Aristaless related homeobox gene (ARX).
Neurobiol Dis 153 105329(2021) 33711494

Xu S, Hayashi Y, Takagishi Y, Itoh M, Murata Y.
Aristaless-related homeobox plays a key role in hyperplasia of the pancreas islet α-like cells in mice deficient in proglucagon-derived peptides.
PLoS One 8(5) e64415(2013) 23671715