Mutation Details for c.3873G>C
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cDNA Name
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c.3873G>C
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Protein Name
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p.Gln1291His
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Exon or Intron
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exon 23
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Legacy Exon or Intron
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exon 20
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Q1291H
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Other Details
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Using the chemical cleavage techniwue and direct sequencing, we have identified a substitution of C for G at position 4005 of CFTR. Since this is a fairly conservative change, and the amino acid at his position is not well conserved bewteen related ABC genes, we do not believe that this alone results in a mutation of CFTR.
It has not been possible to confirm CF in the family where this mutation was identified because the affected child has severe eczema preventing a sweat test being preformed. Recurrent chest infections and a arequirement for Creon tablets have been taken as indicative of CF. No other CF mutation has been identified. An older sibling also carries Q1291H, is genotypically identical at all flanking markers and has had a normal sweat test.
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Contributors
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McIntosh I,
Shrimpton T,
Jones C,
Brock D
1990-07-30
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Institute
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University of Edinburgh
Edinburgh, Scotland
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Submitted Phenotype Details
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Reference
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Jones et al. 1992
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