Screening for partial deletions in the CREBBP gene in Rubinstein-Taybi syndrome patients using multiplex PCR/liquid chromatography

Genet Test. 2006 Winter;10(4):265-71. doi: 10.1089/gte.2006.10.265.

Abstract

Rubinstein-Taybi syndrome (RTS, MIM 180849) is a multiple malformation syndrome characterized by growth retardation, developmental delay, and dysmorphic features, including down-slanting palpebral fissures, a beaked nose, broad thumbs, and halluces. Mutations in the gene encoding the CREB-binding protein gene (CREBBP, also known as CBP) on chromosome 16p13.3 were identified in 1995. Recently, we developed a mutation analysis protocol using denaturing high-performance liquid chromatography (DHPLC) and identified heterozygous CREBBP mutations in 12 of 21 RTS patients. To test whether exonic deletions represent a common pathogenic mechanism, we assessed the copy number of all the coding exons using a recently developed method, the multiplex PCR/liquid chromatography assay (MP/LC). By using MP/LC, we performed screening for CREBBP exonic deletions among 25 RTS patients in whom no point mutations or small insertions/deletions were identified by DHPLC screening. We identified four classic RTS patients with deletions encompassing multiple exons (14-16, 5-31, 1-16, and 4-26). We conclude that large deletions including several exons are a relatively frequent cause of RTS, and that MP/LC is an effective method for detecting these deletions.

Publication types

  • Evaluation Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • CREB-Binding Protein / genetics*
  • Chromatography, High Pressure Liquid*
  • DNA Mutational Analysis
  • Female
  • Gene Deletion*
  • Gene Dosage
  • Genetic Testing / methods*
  • Heteroduplex Analysis
  • Humans
  • Infant
  • Male
  • Polymerase Chain Reaction*
  • Rubinstein-Taybi Syndrome / diagnosis*
  • Rubinstein-Taybi Syndrome / genetics

Substances

  • CREB-Binding Protein
  • CREBBP protein, human