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

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Abstract
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.