B6;129-Ercc6tm1GvhAdh5tm1Stam /H

Status

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EMMA IDEM:14674
Citation informationRRID:IMSR_EM:14674 

Research Resource Identifiers (RRID) are persistent unique ID numbers assigned to help researchers cite key resources (e.g. antibodies, model organisms and software projects) in the biomedical literature to improve transparency and reproducibility in research. See https://www.rrids.org/ for more information.

International strain nameB6;129-Ercc6tm1GvhAdh5tm1Stam /H
Alternative nameCsb/Adh5 double KO B6J.129P2-Ercc6 tm1Gvh Adh5 tm1Stam/H
Strain typeTargeted Mutant Strains : Knock-out
Allele/Transgene symbolErcc6tm1Gvh, Adh5tm1Stam
Gene/Transgene symbolErcc6, Adh5

Information from provider

ProviderMulderrig Lee
Provider affiliationLab of Molecular Biology, MRC
Additional ownerErcc6 (Csb) allele was generated and is owned by Gijsbertus van der Horst lab, Erasmus University, Netherlands. Adh5 was generated and is owned by Jonathan S Stamler’s lab, Duke University Medical Centre, USA.
Genetic informationAdh5: This is a targeted null mutation. Homologous recombination was used to replace exons 5 and 6 with a neomycin resistance gene. The deleted region encoded most of the coenzyme binding domain. S-nitrosoglutathione reductase activity was undetected in various tissues obtained from homozygous mutant mice. Ercc6: This is a targeted null mutation. Insertion of a floxed neomycin resistance cassette into exon 5 near a site corresponding to a known human mutation disrupted the Ercc6 gene. RT-PCR and Western blot analyses of E13.5 fibroblasts from homozygous mutant animals did not detect wild-type transcript or wild-type protein, but indicate that the mutant allele expresses mRNA at low levels.
Phenotypic informationHomozygous:
Homozygous double knock-out mice are small and develop ataxia.

Heterozygous:
To be confirmed.
Breeding historyUnspecified number of backcrosses to C57BL/6J.
References
  • Essential roles of S-nitrosothiols in vascular homeostasis and endotoxic shock.;Liu Limin, Yan Yun, Zeng Ming, Zhang Jian, Hanes Martha A, Ahearn Gregory, McMahon Timothy J, Dickfeld Timm, Marshall Harvey E, Que Loretta G, Stamler Jonathan S, ;2004;Cell;116;617-28; 14980227
  • Aldehyde-driven transcriptional stress triggers an anorexic DNA damage response.;Mulderrig Lee, Garaycoechea Juan I, Tuong Zewen K, Millington Christopher L, Dingler Felix A, Ferdinand John R, Gaul Liam, Tadross John A, Arends Mark J, O'Rahilly Stephen, Crossan Gerry P, Clatworthy Menna R, Patel Ketan J, ;2021;Nature;600;158-163; 34819667
  • Defective transcription-coupled repair in Cockayne syndrome B mice is associated with skin cancer predisposition.;van der Horst G T, van Steeg H, Berg R J, van Gool A J, de Wit J, Weeda G, Morreau H, Beems R B, van Kreijl C F, de Gruijl F R, Bootsma D, Hoeijmakers J H, ;1997;Cell;89;425-35; 9150142
Homozygous fertileyes
Homozygous viableyes
Homozygous matings requiredno
Immunocompromisedunknown

Information from EMMA

Archiving centreMary Lyon Centre at MRC Harwell, Oxford, United Kingdom

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

IMPC phenotypes (gene matching)
  • decreased exploration in new environment / IMPC
  • abnormal vocalization / IMPC
  • decreased spleen weight / IMPC
MGI phenotypes (gene matching)
  • increased susceptibility to bacterial infection / MGI
  • decreased systemic arterial blood pressure / MGI
  • abnormal metabolism / MGI
  • increased physiological sensitivity to xenobiotic / MGI
  • increased sensitivity to xenobiotic induced morbidity/mortality / MGI
  • increased susceptibility to bacterial infection induced morbidity/mortality / MGI

Literature references

  • Essential roles of S-nitrosothiols in vascular homeostasis and endotoxic shock.;Liu Limin, Yan Yun, Zeng Ming, Zhang Jian, Hanes Martha A, Ahearn Gregory, McMahon Timothy J, Dickfeld Timm, Marshall Harvey E, Que Loretta G, Stamler Jonathan S, ;2004;Cell;116;617-28; 14980227
  • Aldehyde-driven transcriptional stress triggers an anorexic DNA damage response.;Mulderrig Lee, Garaycoechea Juan I, Tuong Zewen K, Millington Christopher L, Dingler Felix A, Ferdinand John R, Gaul Liam, Tadross John A, Arends Mark J, O'Rahilly Stephen, Crossan Gerry P, Clatworthy Menna R, Patel Ketan J, ;2021;Nature;600;158-163; 34819667
  • Defective transcription-coupled repair in Cockayne syndrome B mice is associated with skin cancer predisposition.;van der Horst G T, van Steeg H, Berg R J, van Gool A J, de Wit J, Weeda G, Morreau H, Beems R B, van Kreijl C F, de Gruijl F R, Bootsma D, Hoeijmakers J H, ;1997;Cell;89;425-35; 9150142

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