Literature DB >> 1713973

The incidence of different cystic fibrosis mutations in the Scottish population: effects on prenatal diagnosis and genetic counselling.

A E Shrimpton1, I McIntosh, D J Brock.   

Abstract

We present an analysis of the frequency of 16 different cystic fibrosis (CF) mutant alleles in the Scottish population. Each allele was detected in DNA amplified by the polymerase chain reaction (PCR) either directly on polyacrylamide gels, on agarose gels after restriction enzyme digestion, or by using allele specific oligonucleotides. Among 506 CF chromosomes, of predominantly Scottish origin, the frequencies of the different mutations were delta F508 0.71, G551D 0.05, G542X 0.04, R117H 0.01, 1717-1G----A 0.01, A455E + delta I507 + R553X + R560T + W1282X + 621 + 1G----T combined 0.03, unpublished 0.01, and unknown 0.13. No examples of D110H, R347P, S549N, S549I, or 2566ins AT mutations were found. The relevance of this type of analysis for both prenatal diagnosis and heterozygote screening is discussed.

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Year:  1991        PMID: 1713973      PMCID: PMC1016849          DOI: 10.1136/jmg.28.5.317

Source DB:  PubMed          Journal:  J Med Genet        ISSN: 0022-2593            Impact factor:   6.318


  14 in total

1.  A 3' splice site consensus sequence mutation in the cystic fibrosis gene.

Authors:  H Guillermit; P Fanen; C Ferec
Journal:  Hum Genet       Date:  1990-09       Impact factor: 4.132

2.  The haplotype distribution of the delta F508 mutation in cystic fibrosis families in Scotland.

Authors:  I McIntosh; A Curtis; M L Lorenzo; M Keston; A J Gilfillan; G Morris; D J Brock
Journal:  Hum Genet       Date:  1990-09       Impact factor: 4.132

3.  Identification of mutations in regions corresponding to the two putative nucleotide (ATP)-binding folds of the cystic fibrosis gene.

Authors:  B S Kerem; J Zielenski; D Markiewicz; D Bozon; E Gazit; J Yahav; D Kennedy; J R Riordan; F S Collins; J M Rommens
Journal:  Proc Natl Acad Sci U S A       Date:  1990-11       Impact factor: 11.205

4.  Three point mutations in the CFTR gene in French cystic fibrosis patients: identification by denaturing gradient gel electrophoresis.

Authors:  M Vidaud; P Fanen; J Martin; N Ghanem; S Nicolas; M Goossens
Journal:  Hum Genet       Date:  1990-09       Impact factor: 4.132

5.  Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA.

Authors:  J R Riordan; J M Rommens; B Kerem; N Alon; R Rozmahel; Z Grzelczak; J Zielenski; S Lok; N Plavsic; J L Chou
Journal:  Science       Date:  1989-09-08       Impact factor: 47.728

6.  Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells.

Authors:  D P Rich; M P Anderson; R J Gregory; S H Cheng; S Paul; D M Jefferson; J D McCann; K W Klinger; A E Smith; M J Welsh
Journal:  Nature       Date:  1990-09-27       Impact factor: 49.962

7.  A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis conductance regulator protein.

Authors:  G R Cutting; L M Kasch; B J Rosenstein; J Zielenski; L C Tsui; S E Antonarakis; H H Kazazian
Journal:  Nature       Date:  1990-07-26       Impact factor: 49.962

8.  Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer.

Authors:  M L Drumm; H A Pope; W H Cliff; J M Rommens; S A Marvin; L C Tsui; F S Collins; R A Frizzell; J M Wilson
Journal:  Cell       Date:  1990-09-21       Impact factor: 41.582

9.  Polymerase chain reaction for detection of the alpha-1-antitrypsin Z allele in chronic liver disease.

Authors:  A M Brind; I McIntosh; D J Brock; O F James; M F Bassendine
Journal:  J Hepatol       Date:  1990-03       Impact factor: 25.083

10.  A consortium approach to molecular genetic services. Scottish Molecular Genetics Consortium.

Authors:  D J Brock
Journal:  J Med Genet       Date:  1990-01       Impact factor: 6.318

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  13 in total

1.  Cystic fibrosis genotypes and views on screening are both heterogeneous and population related.

Authors:  C R Scriver; T M Fujiwara
Journal:  Am J Hum Genet       Date:  1992-11       Impact factor: 11.025

2.  Mutation analysis of 184 cystic fibrosis families in Wales.

Authors:  J Cheadle; J Myring; L al-Jader; L Meredith
Journal:  J Med Genet       Date:  1992-09       Impact factor: 6.318

3.  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

4.  Evaluation of laboratory methods for cystic fibrosis carrier screening: reliability, sensitivity, specificity, and costs.

Authors:  Z H Miedzybrodzka; Z Yin; K F Kelly; N E Haites
Journal:  J Med Genet       Date:  1994-07       Impact factor: 6.318

5.  Prenatal cystic fibrosis carrier testing: designing an information leaflet to meet the specific needs of the target population.

Authors:  M E Mennie; W A Liston; D J Brock
Journal:  J Med Genet       Date:  1992-05       Impact factor: 6.318

6.  Cost effectiveness of antenatal screening for cystic fibrosis.

Authors:  H S Cuckle; G A Richardson; T A Sheldon; P Quirke
Journal:  BMJ       Date:  1995-12-02

7.  Prevalence of cystic fibrosis mutations in the Grampian region of Scotland.

Authors:  Z H Miedzybrodzka; J C Dean; G Russell; J A Friend; K F Kelly; N E Haites
Journal:  J Med Genet       Date:  1993-04       Impact factor: 6.318

8.  Antenatal screening for carriers of cystic fibrosis: randomised trial of stepwise v couple screening.

Authors:  Z H Miedzybrodzka; M H Hall; J Mollison; A Templeton; I T Russell; J C Dean; K F Kelly; T M Marteau; N E Haites
Journal:  BMJ       Date:  1995-02-11

9.  Uptake of cystic fibrosis testing in primary care: supply push or demand pull?

Authors:  H Bekker; M Modell; G Denniss; A Silver; C Mathew; M Bobrow; T Marteau
Journal:  BMJ       Date:  1993-06-12

10.  Identification of the M1101K mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and complete detection of cystic fibrosis mutations in the Hutterite population.

Authors:  J Zielenski; T M Fujiwara; D Markiewicz; A J Paradis; A I Anacleto; B Richards; R H Schwartz; K W Klinger; L C Tsui; K Morgan
Journal:  Am J Hum Genet       Date:  1993-03       Impact factor: 11.025

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