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Article: Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor

TitleModulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor
Authors
Issue Date1996
PublisherNature Publishing Group. The Journal's web site is located at http://www.genetics.nature.com
Citation
Nature Genetics, 1996, v. 12 n. 3, p. 280-287 How to Cite?
Abstract
Mice that have been made deficient for the cystic fibrosis transmembrane conductance regulator (Cftr) usually die of intestinal obstruction. We have created Cftr-deficient mice and demonstrate prolonged survival among backcross and intercross progeny with different inbred strains, suggesting that modulation of disease severity is genetically determined. A genome scan showed that the major modifier locus maps near the centromere of mouse chromosome 7. Electrophysiological studies on mice with prolonged survival show that the partial rectification of Cl- and Na+ ion transport abnormalities can be explained in part by up-regulation of a calcium- activated Cl- conductance. Identification of modifier genes in our Cftr(m1HSC)/Cftr(m1HSC) mice should provide important insight into the heterogeneous disease presentation observed among CF patients.
DescriptionErratum in Nature Genetics, 1996, v. 13 n. 1, p. 129
Persistent Identifierhttp://hdl.handle.net/10722/44297
ISSN
2013 Impact Factor: 29.648
2013 SCImago Journal Rankings: 24.052
ISI Accession Number ID
References

 

Author Affiliations
  1. University of Toronto Faculty of Medicine
  2. University of Toronto
  3. Hospital for Sick Children University of Toronto
  4. McGill University
DC FieldValueLanguage
dc.contributor.authorRozmahel, Ren_HK
dc.contributor.authorWilschanski, Men_HK
dc.contributor.authorMatin, Aen_HK
dc.contributor.authorPlyte, Sen_HK
dc.contributor.authorOliver, Men_HK
dc.contributor.authorAuerbach, Wen_HK
dc.contributor.authorMoore, Aen_HK
dc.contributor.authorForstner, Jen_HK
dc.contributor.authorDurie, Pen_HK
dc.contributor.authorNadeau, Jen_HK
dc.contributor.authorBear, Cen_HK
dc.contributor.authorTsui, LCen_HK
dc.date.accessioned2007-09-12T03:50:52Z-
dc.date.available2007-09-12T03:50:52Z-
dc.date.issued1996en_HK
dc.identifier.citationNature Genetics, 1996, v. 12 n. 3, p. 280-287en_HK
dc.identifier.issn1061-4036en_HK
dc.identifier.urihttp://hdl.handle.net/10722/44297-
dc.descriptionErratum in Nature Genetics, 1996, v. 13 n. 1, p. 129-
dc.description.abstractMice that have been made deficient for the cystic fibrosis transmembrane conductance regulator (Cftr) usually die of intestinal obstruction. We have created Cftr-deficient mice and demonstrate prolonged survival among backcross and intercross progeny with different inbred strains, suggesting that modulation of disease severity is genetically determined. A genome scan showed that the major modifier locus maps near the centromere of mouse chromosome 7. Electrophysiological studies on mice with prolonged survival show that the partial rectification of Cl- and Na+ ion transport abnormalities can be explained in part by up-regulation of a calcium- activated Cl- conductance. Identification of modifier genes in our Cftr(m1HSC)/Cftr(m1HSC) mice should provide important insight into the heterogeneous disease presentation observed among CF patients.en_HK
dc.languageengen_HK
dc.publisherNature Publishing Group. The Journal's web site is located at http://www.genetics.nature.comen_HK
dc.relation.ispartofNature Geneticsen_HK
dc.subject.meshChlorides - metabolismen_HK
dc.subject.meshCystic fibrosis - genetics - pathology - physiopathologyen_HK
dc.subject.meshCystic fibrosis transmembrane conductance regulator - deficiency - geneticsen_HK
dc.subject.meshIleum - pathologyen_HK
dc.subject.meshDisease models, animalen_HK
dc.titleModulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factoren_HK
dc.typeArticleen_HK
dc.identifier.emailTsui, LC: tsuilc@hkucc.hku.hken_HK
dc.identifier.authorityTsui, LC=rp00058en_HK
dc.description.natureabstracten_HK
dc.identifier.doi10.1038/ng0396-280en_HK
dc.identifier.pmid8589719en_HK
dc.identifier.scopuseid_2-s2.0-13344282728en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-13344282728&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume12en_HK
dc.identifier.issue3en_HK
dc.identifier.spage280en_HK
dc.identifier.epage287en_HK
dc.identifier.isiWOS:A1996TY18300017-
dc.publisher.placeUnited Statesen_HK
dc.identifier.scopusauthoridRozmahel, R=6701510561en_HK
dc.identifier.scopusauthoridWilschanski, M=6701812857en_HK
dc.identifier.scopusauthoridMatin, A=35581268900en_HK
dc.identifier.scopusauthoridPlyte, S=6701650532en_HK
dc.identifier.scopusauthoridOliver, M=16157886700en_HK
dc.identifier.scopusauthoridAuerbach, W=6701322806en_HK
dc.identifier.scopusauthoridMoore, A=7402762852en_HK
dc.identifier.scopusauthoridForstner, J=7006092197en_HK
dc.identifier.scopusauthoridDurie, P=7005360997en_HK
dc.identifier.scopusauthoridNadeau, J=7102010316en_HK
dc.identifier.scopusauthoridBear, C=7006718679en_HK
dc.identifier.scopusauthoridTsui, LC=7102754167en_HK

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