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.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
MeSH terms
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Anemia, Diamond-Blackfan / genetics*
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Anemia, Diamond-Blackfan / metabolism*
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Cell Line
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Down-Regulation
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Humans
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Lymphocytes / metabolism
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Models, Biological
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Mutation
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RNA Processing, Post-Transcriptional
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RNA, Ribosomal, 18S / genetics*
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RNA, Ribosomal, 18S / metabolism*
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RNA, Ribosomal, 28S / genetics
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RNA, Ribosomal, 28S / metabolism
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RNA, Small Interfering / genetics
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Ribosomal Proteins / antagonists & inhibitors
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Ribosomal Proteins / chemistry
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Ribosomal Proteins / deficiency
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Ribosomal Proteins / genetics*
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Ribosomal Proteins / metabolism*
Substances
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RNA, Ribosomal, 18S
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RNA, Ribosomal, 28S
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RNA, Small Interfering
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Ribosomal Proteins
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ribosomal protein S19