Literature DB >> 15017334

Frequency of the cystic fibrosis 3199del6 mutation in individuals heterozygous for I148T.

Arlene Buller1, Susan Olson, Joy B Redman, Feras Hantash, Rebecca Chen, Charles M Strom.   

Abstract

PURPOSE: To determine the carrier frequency of the 3199del6 cystic fibrosis (CF) mutation in individuals heterozygous for I148T in a large-scale CF testing population.
METHODS: DNA samples from 439 consecutive I148T-heterozygous individuals were screened for the 3199del6 mutation using a laboratory-developed test.
RESULTS: Genotyping revealed four samples heterozygous for the 3199del6 mutation (0.9%). The four samples positive for 3199del6 had an IVS 8 genotype of 7T/9T. The 3199del6 mutation was not observed after genotyping of 348 random, anonymous samples.
CONCLUSION: The 3199del6 mutation occurs in 0.9% of individuals positive for the I148T mutation and <0.07% of chromosomes that are wild type for the ACMG panel mutations.

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Year:  2004        PMID: 15017334     DOI: 10.1097/01.gim.0000117332.18002.ff

Source DB:  PubMed          Journal:  Genet Med        ISSN: 1098-3600            Impact factor:   8.822


  7 in total

1.  Best practice guidelines for molecular genetic diagnosis of cystic fibrosis and CFTR-related disorders--updated European recommendations.

Authors:  Els Dequeker; Manfred Stuhrmann; Michael A Morris; Teresa Casals; Carlo Castellani; Mireille Claustres; Harry Cuppens; Marie des Georges; Claude Ferec; Milan Macek; Pier-Franco Pignatti; Hans Scheffer; Marianne Schwartz; Michal Witt; Martin Schwarz; Emmanuelle Girodon
Journal:  Eur J Hum Genet       Date:  2008-08-06       Impact factor: 4.246

2.  Molecular testing for cystic fibrosis carrier status practice guidelines: recommendations of the National Society of Genetic Counselors.

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3.  Technical validation of a TM Biosciences Luminex-based multiplex assay for detecting the American College of Medical Genetics recommended cystic fibrosis mutation panel.

Authors:  Charles M Strom; Richard Janeszco; Franklin Quan; Sheng-biao Wang; Arlene Buller; Matthew McGinniss; Weimin Sun
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4.  Cystic fibrosis prenatal screening in genetic counseling practice: recommendations of the National Society of Genetic Counselors.

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Journal:  J Genet Couns       Date:  2005-02       Impact factor: 2.537

Review 5.  Consensus on the use and interpretation of cystic fibrosis mutation analysis in clinical practice.

Authors:  C Castellani; H Cuppens; M Macek; J J Cassiman; E Kerem; P Durie; E Tullis; B M Assael; C Bombieri; A Brown; T Casals; M Claustres; G R Cutting; E Dequeker; J Dodge; I Doull; P Farrell; C Ferec; E Girodon; M Johannesson; B Kerem; M Knowles; A Munck; P F Pignatti; D Radojkovic; P Rizzotti; M Schwarz; M Stuhrmann; M Tzetis; J Zielenski; J S Elborn
Journal:  J Cyst Fibros       Date:  2008-05       Impact factor: 5.482

6.  Phenotyping Rare CFTR Mutations Reveal Functional Expression Defects Restored by TRIKAFTATM.

Authors:  Onofrio Laselva; Maria C Ardelean; Christine E Bear
Journal:  J Pers Med       Date:  2021-04-15

7.  G378X-I148T CFTR variant: A new complex allele in a cystic fibrosis newborn with pancreatic insufficiency.

Authors:  Vito Terlizzi; Claudia Centrone; Matteo Botti; Giovanni Taccetti
Journal:  Mol Genet Genomic Med       Date:  2022-08-13       Impact factor: 2.473

  7 in total

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