Quantitative variation in cystic fibrosis-associated proteins in cystic fibrosis patients, carriers, and controls

Jamieson, Andrew; Mackinlay, Elizabeth E.; Aitken, David A.; Cooke, Allan R.; Ferguson‐Smith, M. A.

doi:10.1007/bf00273076

Hum Genet

1985

DOI: 10.1007/bf00273076

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Quantitative variation in cystic fibrosis-associated proteins in cystic fibrosis patients, carriers, and controls

Andrew Jamieson

Elizabeth E. Mackinlay

David A. Aitken

et al.

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1986

1989

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Cited by 3 publications

References 16 publications

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Cystic fibrosis proteins detected by electrophoretic techniques

Guy-Crotte¹,

Barthe²,

Figarella³

1989

Electrophoresis

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For the detection of the cystic fibrosis protein (CFP) in serum of cystic fibrosis (CF) carriers, thin-layer polyacrylamide gel isoelectric focusing proved inappropriate as a diagnostic test, but was useful for screening fractions on purification of CFP by chromatofocusing on a Mono P column. On sodium dodecyl sulfate-polyacrylamide gel electrophoresis an Mr 12,000 protein (P12) was found in most CFP-positive sera, indicating good correlation between these two CF-associated proteins. Detection of the P12 protein by sodium dodecyl sulfate-polyacrylamide gel electrophoresis was well reproducible and less delicate than IEF. The technique was also used to purify P12 from serum by two successive preparative electrophoresis steps in a 7.5-15% gradient and 15% homogeneous gel. The use of silver staining revealed that P12, which was present in all sera of CF patients and carriers with variable intensities, was also present in trace amounts in normal sera.

show abstract

Cystic fibrosis proteins detected by electrophoretic techniques

Guy-Crotte¹,

Barthe²,

Figarella³

1989

Electrophoresis

View full text Add to dashboard Cite

show abstract

Identification of cystic fibrosis homozygotes and heterozygotes by isoelectric focussing of serum proteins

Getliffe

Ward

Shepherd

1986

J of Inher Metab Disea

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Screening of 44 cystic fibrosis homozygotes, 17 heterozygotes and 36 normal controls, by identification of cystic fibrosis protein (CFP), was performed on sera using an improved isoelectric focusing technique. CFP was observed in 91% of homozygotes, 88% of heterozygotes and 8% of normal controls tested. Partial purification of CFP by chromatofocussing indicates that CFP has a molecular weight of about 52,000 u. It is speculated that CFP is a normal serum protein that exhibits a quantitative difference in concentration between CF homozygotes, heterozygotes and normals.

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Correlation between the cystic fibrosis protein detected by isoelectric focusing and a protein of 12 kDa detected by SDS-polyacrylamide gel electrophoresis

Barthe¹,

Figarella²,

Galabert

et al. 1987

Clinica Chimica Acta

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Quantitative variation in cystic fibrosis-associated proteins in cystic fibrosis patients, carriers, and controls

Cited by 3 publications

References 16 publications

Cystic fibrosis proteins detected by electrophoretic techniques

Cystic fibrosis proteins detected by electrophoretic techniques

Identification of cystic fibrosis homozygotes and heterozygotes by isoelectric focussing of serum proteins

Correlation between the cystic fibrosis protein detected by isoelectric focusing and a protein of 12 kDa detected by SDS-polyacrylamide gel electrophoresis

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