B6.129(Cg)-Hrastm2Xbr/Cnbc

Status

Available to order

EMMA IDEM:05942
Citation informationRRID:IMSR_EM:05942 

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International strain nameB6.129(Cg)-Hrastm2Xbr/Cnbc
Alternative nameHras1
Strain typeTargeted Mutant Strains : Conditional mutation
Allele/Transgene symbolHrastm2Xbr
Gene/Transgene symbolHras

Information from provider

ProviderMariano Barbacid
Provider affiliationMolecular Oncology, Centro Nacional de Investigaciones Oncologicas
Genetic informationIn ES cells containing Hras1tm1Xbr, in which an IRES-beta-geo cassette was integrated into the 3'-UTR of the Hras gene, a floxed hygro STOP cassette was inserted upstream of coding exon 1. In addition, a G to T substitution was created at the second base of codon 12 of exon 1. This results in an amino acid substitution of valine for glycine at position 12. Upon cre-mediated recombination, removal of the hygro STOP cassette leads to the expression of the HrasG12V mutation and the IRES-beta-geo cassette so that the lacZ reporter is expressed in those cells that express the mutated HrasG12V gene.
Phenotypic informationBefore cre-mediated recombination there is no phenotype associated to this allele. After germ-line cre-mediated excision of the floxed STOP hygro cassette, expression of the HrasG12V mutant leads to multiple abnormalities, mostly related to craniofacial, cardiovascular and mammary gland development and tumorigenesis. This is a mouse model of human Costello syndrome.
Breeding historyChimeras were crossed with CD1 females for germ line transmission analysis. Heterozygous offspring were crossed among themselves to generate homozygous mutants. Heterozygous animals were backcrossed to C57BL/6J for more than 10 generations. After cre-mediated recombination homozygous mice HrasG12V/G12V are viable and fertile.
References
  • A mouse model for Costello syndrome reveals an Ang II-mediated hypertensive condition.;Schuhmacher Alberto J, Guerra Carmen, Sauzeau Vincent, Cañamero Marta, Bustelo Xosé R, Barbacid Mariano, ;2008;The Journal of clinical investigation;118;2169-79; 18483625
  • Germline expression of H-Ras(G12V) causes neurological deficits associated to Costello syndrome.;Viosca J, Schuhmacher A J, Guerra C, Barco A, ;2009;Genes, brain, and behavior;8;60-71; 18823404
Homozygous fertileyes
Homozygous viableyes
Homozygous matings requiredno
Immunocompromisedno

Information from EMMA

Archiving centreCNB-CSIC, Centro Nacional de Biotecnologia, Madrid, Spain
Animals used for archivingheterozygous C57BL/6J males

Disease and phenotype information

MGI allele-associated human disease models

Orphanet associated rare diseases, based on orthologous gene matching

MGI phenotypes (allele matching)
  • short premaxilla / MGI
  • short maxilla / MGI
  • abnormal frontal bone morphology / MGI
  • hypertension / MGI
  • abnormal heart morphology / MGI
  • heart right ventricle hypertrophy / MGI
  • abnormal cranium morphology / MGI
  • abnormal mammary gland development / MGI
  • mammary gland hyperplasia / MGI
  • abnormal branching of the mammary ductal tree / MGI
  • abnormal blood vessel morphology / MGI
  • increased mammary adenocarcinoma incidence / MGI
  • neoplasm / MGI
  • heart left ventricle hypertrophy / MGI
  • increased systemic arterial blood pressure / MGI
  • short zygomatic bone / MGI
  • abnormal facial morphology / MGI
  • abnormal lip morphology / MGI
  • renal fibrosis / MGI
  • renal/urinary system phenotype / MGI
  • cardiovascular system phenotype / MGI
  • respiratory system phenotype / MGI
  • abnormal circulating protein level / MGI
  • increased systemic arterial diastolic blood pressure / MGI
  • increased systemic arterial systolic blood pressure / MGI
  • enlarged heart atrium / MGI
  • increased heart ventricle size / MGI
  • abnormal aorta tunica media morphology / MGI
  • abnormal vibrissa follicle morphology / MGI
  • enlarged aortic valve / MGI
  • decreased urine creatinine level / MGI
  • depressed nasal bridge / MGI
  • short nasal bridge / MGI
  • choanal stenosis / MGI
MGI phenotypes (gene matching)
  • short premaxilla / MGI
  • short maxilla / MGI
  • abnormal frontal bone morphology / MGI
  • hypertension / MGI
  • abnormal heart morphology / MGI
  • heart right ventricle hypertrophy / MGI
  • abnormal cranium morphology / MGI
  • abnormal mammary gland development / MGI
  • mammary gland hyperplasia / MGI
  • abnormal branching of the mammary ductal tree / MGI
  • abnormal blood vessel morphology / MGI
  • increased mammary adenocarcinoma incidence / MGI
  • neoplasm / MGI
  • no abnormal phenotype detected / MGI
  • heart left ventricle hypertrophy / MGI
  • increased systemic arterial blood pressure / MGI
  • no phenotypic analysis / MGI
  • short zygomatic bone / MGI
  • nervous system phenotype / MGI
  • abnormal facial morphology / MGI
  • abnormal lip morphology / MGI
  • renal fibrosis / MGI
  • decreased incidence of tumors by chemical induction / MGI
  • renal/urinary system phenotype / MGI
  • cardiovascular system phenotype / MGI
  • respiratory system phenotype / MGI
  • abnormal circulating protein level / MGI
  • increased systemic arterial diastolic blood pressure / MGI
  • increased systemic arterial systolic blood pressure / MGI
  • enlarged heart atrium / MGI
  • increased heart ventricle size / MGI
  • abnormal aorta tunica media morphology / MGI
  • abnormal vibrissa follicle morphology / MGI
  • enlarged aortic valve / MGI
  • decreased urine creatinine level / MGI
  • chylous ascites / MGI
  • depressed nasal bridge / MGI
  • short nasal bridge / MGI
  • choanal stenosis / MGI

Literature references

  • A mouse model for Costello syndrome reveals an Ang II-mediated hypertensive condition.;Schuhmacher Alberto J, Guerra Carmen, Sauzeau Vincent, Cañamero Marta, Bustelo Xosé R, Barbacid Mariano, ;2008;The Journal of clinical investigation;118;2169-79; 18483625
  • Germline expression of H-Ras(G12V) causes neurological deficits associated to Costello syndrome.;Viosca J, Schuhmacher A J, Guerra C, Barco A, ;2009;Genes, brain, and behavior;8;60-71; 18823404

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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*
  • Frozen sperm. Delivered in 4 weeks (after paperwork in place). €1740*
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Practical information

Genotyping protocol

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