Literature DB >> 9155619

Absence of mutations in the regulatory domain of the gap junction protein connexin 43 in patients with visceroatrial heterotaxy.

M Penman Splitt1, M Y Tsai, J Burn, J A Goodship.   

Abstract

OBJECTIVE: To determine the frequency of mutations in the regulatory domain of the gap junction protein connexin 43 in patients with visceroatrial heterotaxy.
DESIGN: Mutation screening of the terminal 200 base pairs of connexin43 gene coding sequence in a series of patients from tertiary care centres. PATIENTS: 48 patients with visceroatrial heterotaxy attending UK Regional Paediatric Cardiology Centres.
RESULTS: No changes from the published connexin43 consensus sequence were found in any of the 48 patients studied.
CONCLUSIONS: Germline mutations of the phosphorylation sites in teh regulatory domain of the connexin43 gene are rare in patients with visceroatrial heterotaxy.

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Year:  1997        PMID: 9155619      PMCID: PMC484734          DOI: 10.1136/hrt.77.4.369

Source DB:  PubMed          Journal:  Heart        ISSN: 1355-6037            Impact factor:   5.994


  6 in total

1.  Connexin 43 expression in the mouse embryo: localization of transcripts within developmentally significant domains.

Authors:  C P Ruangvoravat; C W Lo
Journal:  Dev Dyn       Date:  1992-08       Impact factor: 3.780

2.  The human connexin gene family of gap junction proteins: distinct chromosomal locations but similar structures.

Authors:  G I Fishman; R L Eddy; T B Shows; L Rosenthal; L A Leinwand
Journal:  Genomics       Date:  1991-05       Impact factor: 5.736

3.  Failure to detect connexin43 mutations in 38 cases of sporadic and familial heterotaxy.

Authors:  M Gebbia; J A Towbin; B Casey
Journal:  Circulation       Date:  1996-10-15       Impact factor: 29.690

4.  Mutations of the Connexin43 gap-junction gene in patients with heart malformations and defects of laterality.

Authors:  S H Britz-Cunningham; M M Shah; C W Zuppan; W H Fletcher
Journal:  N Engl J Med       Date:  1995-05-18       Impact factor: 91.245

5.  Cardiac malformation in neonatal mice lacking connexin43.

Authors:  A G Reaume; P A de Sousa; S Kulkarni; B L Langille; D Zhu; T C Davies; S C Juneja; G M Kidder; J Rossant
Journal:  Science       Date:  1995-03-24       Impact factor: 47.728

6.  Connexin43 mutations in sporadic and familial defects of laterality.

Authors:  B Casey; A Ballabio
Journal:  N Engl J Med       Date:  1995-10-05       Impact factor: 91.245

  6 in total
  4 in total

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Review 2.  Temporal regulation of connexin phosphorylation in embryonic and adult tissues.

Authors:  Timothy J King; Paul D Lampe
Journal:  Biochim Biophys Acta       Date:  2005-08-08

3.  A submicroscopic deletion in Xq26 associated with familial situs ambiguus.

Authors:  G B Ferrero; M Gebbia; G Pilia; D Witte; A Peier; R J Hopkin; W J Craigen; L G Shaffer; D Schlessinger; A Ballabio; B Casey
Journal:  Am J Hum Genet       Date:  1997-08       Impact factor: 11.025

4.  Evaluating the role of connexin43 in congenital heart disease: Screening for mutations in patients with outflow tract anomalies and the analysis of knock-in mouse models.

Authors:  Guo-Ying Huang; Li-Jian Xie; Kaari L Linask; Chen Zhang; Xiao-Qing Zhao; Yi Yang; Guo-Min Zhou; Ying-Jie Wu; Lucrecia Marquez-Rosado; Doff B McElhinney; Elizabeth Goldmuntz; Chengyu Liu; Paul D Lampe; Bishwanath Chatterjee; Cecilia W Lo
Journal:  J Cardiovasc Dis Res       Date:  2011-10
  4 in total

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