Gene expression profiling of CD34+ cells in patients with the 5q- syndrome

Br J Haematol. 2007 Nov;139(4):578-89. doi: 10.1111/j.1365-2141.2007.06833.x. Epub 2007 Oct 3.

Abstract

The transcriptome of the CD34+ cells was determined in a group of 10 patients with the 5q- syndrome using a comprehensive array platform, and was compared with the transcriptome of CD34+ cells from 16 healthy control subjects and 14 patients with refractory anaemia and a normal karyotype. The majority of the genes assigned to the commonly deleted region (CDR) of the 5q- syndrome at 5q31-q32 showed a reduction in expression levels in patients with the 5q- syndrome, consistent with the loss of one allele. Candidate genes showing haploinsufficiency in the 5q- syndrome included the tumour suppressor gene SPARC and RPS14, a component of the 40S ribosomal subunit. Two genes mapping to the CDR, RBM22 and CSNK1A1, showed a >50% reduction in gene expression, consistent with the downregulation of the remaining allele. This study identified several significantly deregulated gene pathways in patients with the 5q- syndrome and gene pathway analysis data supports the proposal that SPARC may play a role in the pathogenesis of the 5q- syndrome. This study suggests that several of the genes mapping to the CDR of the 5q- syndrome play a role in the pathogenesis of this disorder.

MeSH terms

  • Anemia / genetics*
  • Antigens, CD34 / genetics*
  • Case-Control Studies
  • Chromosome Deletion*
  • Chromosomes, Human, Pair 5 / genetics*
  • Chronic Disease
  • Down-Regulation
  • Gene Expression / genetics*
  • Gene Expression Profiling / methods
  • Humans
  • Myelodysplastic Syndromes / genetics*
  • Reverse Transcriptase Polymerase Chain Reaction / methods
  • Syndrome

Substances

  • Antigens, CD34