Posttranscriptional down-regulation of small ribosomal subunit proteins correlates with reduction of 18S rRNA in RPS19 deficiency

FEBS Lett. 2009 Jun 18;583(12):2049-53. doi: 10.1016/j.febslet.2009.05.023. Epub 2009 May 18.


Ribosomal protein S19 (RPS19) is mutated in patients with Diamond-Blackfan anemia (DBA). We hypothesized that decreased levels of RPS19 lead to a coordinated down-regulation of other ribosomal (r-)proteins at the subunit level. We show that small interfering RNA (siRNA) knock-down of RPS19 results in a relative decrease of small subunit (SSU) r-proteins (S20, S21 and S24) when compared to large subunit (LSU) r-proteins (L3, L9, L30 and L38). This correlates with a relative decrease in 18S rRNA with respect to 28S rRNA. The r-protein mRNA levels remain relatively unchanged indicating a post transcriptional regulation of r-proteins at the level of subunit formation.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Anemia, Diamond-Blackfan / genetics*
  • Anemia, Diamond-Blackfan / metabolism*
  • Cell Line
  • Down-Regulation
  • Humans
  • Lymphocytes / metabolism
  • Models, Biological
  • Mutation
  • RNA Processing, Post-Transcriptional
  • RNA, Ribosomal, 18S / genetics*
  • RNA, Ribosomal, 18S / metabolism*
  • RNA, Ribosomal, 28S / genetics
  • RNA, Ribosomal, 28S / metabolism
  • RNA, Small Interfering / genetics
  • Ribosomal Proteins / antagonists & inhibitors
  • Ribosomal Proteins / chemistry
  • Ribosomal Proteins / deficiency
  • Ribosomal Proteins / genetics*
  • Ribosomal Proteins / metabolism*


  • RNA, Ribosomal, 18S
  • RNA, Ribosomal, 28S
  • RNA, Small Interfering
  • Ribosomal Proteins
  • ribosomal protein S19