Literature DB >> 18383112

Recombination as a mechanism for sporadic mutation in the surfactant protein-C gene.

Amy D McBee1, Daniel J Wegner, Christopher S Carlson, Jennifer A Wambach, Ping Yang, Hillary B Heins, Ola D Saugstad, Michelle A Trusgnich, Julie Watkins-Torry, Lawrence M Nogee, Howard Henderson, F Sessions Cole, Aaron Hamvas.   

Abstract

OBJECTIVE: To determine haplotype background of common mutations in the genes encoding surfactant proteins B and C (SFTPB and SFTPC) and to assess recombination in SFTPC. STUDY
DESIGN: Using comprehensive resequencing of SFTPC and SFTPB, we assessed linkage disequilibrium (LD) (D'), and computationally inferred haplotypes. We computed average recombination rates and Bayes factors (BFs) within SFTPC in a population cohort and near SFTPC (+/-50 kb) in HapMap cohorts. We then biochemically confirmed haplotypes in families with sporadic SFTPC mutations (n = 11) and in individuals with the common SFTPB mutation (121ins2, n = 30).
RESULTS: We detected strong evidence (weak LD and BFs > 1,400) for an intragenic recombination hot spot in both genes. The 121ins2 SFTPB mutation occurred predominantly (89%) on 2 common haplotypes. In contrast, no consistent haplotypes were associated with mutated SFTPC alleles. Sporadic SFTPC mutations arose on the paternal allele in four of five families; the remaining child had evidence for somatic recombination on the mutated allele.
CONCLUSIONS: In contrast to SFTPB, disease alleles at SFTPC do not share a common haplotype background. Most sporadic mutations in SFTPC occurred on the paternal allele, but somatic recombination may be an important mechanism of mutation in SFTPC. Copyright 2008 Wiley-Liss, Inc.

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Year:  2008        PMID: 18383112      PMCID: PMC2765708          DOI: 10.1002/ppul.20782

Source DB:  PubMed          Journal:  Pediatr Pulmonol        ISSN: 1099-0496


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