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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?
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.
Persistent Identifierhttp://hdl.handle.net/10722/44297
ISSN
2014 Impact Factor: 29.352
2014 SCImago Journal Rankings: 19.545
ISI Accession Number ID
References
Errata

 

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.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.naturelink_to_subscribed_fulltexten_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.relation.erratumeid:eid_2-s2.0-0030000483-
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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