A ribosome-related signature in peripheral blood CLL B cells is linked to reduced survival following treatment

Cell Death Dis. 2016 Jun 2;7(6):e2249. doi: 10.1038/cddis.2016.148.

Abstract

We have used polysome profiling coupled to microarray analysis to examine the translatome of a panel of peripheral blood (PB) B cells isolated from 34 chronic lymphocytic leukaemia (CLL) patients. We have identified a 'ribosome-related' signature in CLL patients with mRNAs encoding for ribosomal proteins and factors that modify ribosomal RNA, e.g. DKC1 (which encodes dyskerin, a pseudouridine synthase), showing reduced polysomal association and decreased expression of the corresponding proteins. Our data suggest a general impact of dyskerin dysregulation on the translational apparatus in CLL and importantly patients with low dyskerin levels have a significantly shorter period of overall survival following treatment. Thus, translational dysregulation of dyskerin could constitute a mechanism by which the CLL PB B cells acquire an aggressive phenotype and thus have a major role in oncogenesis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cell Cycle Proteins / genetics
  • Cell Cycle Proteins / metabolism
  • Cell Nucleolus / metabolism
  • Down-Regulation / genetics
  • Eukaryotic Initiation Factors / genetics
  • Eukaryotic Initiation Factors / metabolism
  • Gene Expression Profiling*
  • Gene Expression Regulation, Leukemic
  • Humans
  • Immunoblotting
  • Leukemia, Lymphocytic, Chronic, B-Cell / blood*
  • Leukemia, Lymphocytic, Chronic, B-Cell / drug therapy
  • Leukemia, Lymphocytic, Chronic, B-Cell / genetics*
  • Leukemia, Lymphocytic, Chronic, B-Cell / pathology
  • Nuclear Proteins / genetics
  • Nuclear Proteins / metabolism
  • Polyribosomes / metabolism
  • Protein Biosynthesis
  • RNA, Ribosomal / metabolism
  • Ribosomal Proteins / genetics
  • Ribosomal Proteins / metabolism
  • Ribosomes / metabolism*
  • Survival Analysis
  • Treatment Outcome

Substances

  • Cell Cycle Proteins
  • DKC1 protein, human
  • Eukaryotic Initiation Factors
  • Nuclear Proteins
  • RNA, Ribosomal
  • Ribosomal Proteins