Allelic drop-out in the LDLR gene affects mutation detection in familial hypercholesterolemia

Clin Biochem. 2008 Jan;41(1-2):38-40. doi: 10.1016/j.clinbiochem.2007.09.017. Epub 2007 Oct 11.

Abstract

Objectives: Familial hypercholesterolemia is a monogenic disorder caused by mutations in the LDL receptor (LDLR) gene. We observed allelic drop-out during LDLR genotyping and aimed at redesigning mutation detection.

Design and methods: The NanoChip microelectronic array technology and PCR restriction fragment length polymorphism analysis were used.

Results: Allele drop-out caused false homozygous diagnoses and was overcome using PCR primers without polymorphisms in the primer binding site.

Conclusions: This report presents the importance of allele drop-out in LDLR genotyping.

MeSH terms

  • Alleles*
  • DNA Mutational Analysis* / methods
  • False Positive Reactions
  • Gene Frequency
  • Genetic Carrier Screening
  • Genotype
  • Humans
  • Hyperlipoproteinemia Type II / genetics*
  • Linkage Disequilibrium
  • Mutation*
  • Polymorphism, Single Nucleotide
  • Receptors, LDL / genetics*
  • Research Design

Substances

  • Receptors, LDL