A "silent" polymorphism in the MDR1 gene changes substrate specificity

Science. 2007 Jan 26;315(5811):525-8. doi: 10.1126/science.1135308. Epub 2006 Dec 21.

Abstract

Synonymous single-nucleotide polymorphisms (SNPs) do not produce altered coding sequences, and therefore they are not expected to change the function of the protein in which they occur. We report that a synonymous SNP in the Multidrug Resistance 1 (MDR1) gene, part of a haplotype previously linked to altered function of the MDR1 gene product P-glycoprotein (P-gp), nonetheless results in P-gp with altered drug and inhibitor interactions. Similar mRNA and protein levels, but altered conformations, were found for wild-type and polymorphic P-gp. We hypothesize that the presence of a rare codon, marked by the synonymous polymorphism, affects the timing of cotranslational folding and insertion of P-gp into the membrane, thereby altering the structure of substrate and inhibitor interaction sites.

Publication types

  • Research Support, N.I.H., Intramural

MeSH terms

  • ATP Binding Cassette Transporter, Subfamily B, Member 1 / antagonists & inhibitors
  • ATP Binding Cassette Transporter, Subfamily B, Member 1 / chemistry*
  • ATP Binding Cassette Transporter, Subfamily B, Member 1 / genetics
  • ATP Binding Cassette Transporter, Subfamily B, Member 1 / metabolism*
  • Animals
  • Cell Line
  • Cell Membrane / metabolism
  • Chlorocebus aethiops
  • Codon
  • Cyclosporine / pharmacology
  • Genes, MDR*
  • Haplotypes
  • HeLa Cells
  • Humans
  • Mutagenesis, Site-Directed
  • Polymorphism, Single Nucleotide*
  • Protein Biosynthesis
  • Protein Conformation
  • Protein Folding*
  • Protein Structure, Tertiary
  • RNA, Messenger / genetics
  • RNA, Messenger / metabolism
  • Reverse Transcriptase Polymerase Chain Reaction
  • Rhodamine 123 / metabolism
  • Rhodamine 123 / pharmacology
  • Sirolimus / pharmacology
  • Substrate Specificity
  • Transfection
  • Verapamil / metabolism
  • Verapamil / pharmacology

Substances

  • ATP Binding Cassette Transporter, Subfamily B, Member 1
  • Codon
  • RNA, Messenger
  • Rhodamine 123
  • Cyclosporine
  • Verapamil
  • Sirolimus