title: Posttranscriptional down-regulation of small ribosomal subunit proteins correlates with reduction of 18S rRNA in RPS19 deficiency.
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.
authors.affiliation: Department of Genetics and Pathology, The Rudbeck Laboratory, Uppsala University, Uppsala, Sweden.
authors.forename: Jitendra
authors.initials: J
authors.lastname: Badhai
authors.forename: Anne-Sophie
authors.initials: AS
authors.lastname: Fröjmark
authors.forename: Hamid Reza
authors.initials: HR
authors.lastname: Razzaghian
authors.forename: Edward
authors.initials: E
authors.lastname: Davey
authors.forename: Jens
authors.initials: J
authors.lastname: Schuster
authors.forename: Niklas
authors.initials: N
authors.lastname: Dahl
mesh_terms: D029503:Anemia, Diamond-Blackfan / Q000235:genetics* / Q000378:metabolism*; D002460:Cell Line; D015536:Down-Regulation; D006801:Humans; D008214:Lymphocytes / Q000378:metabolism; D008954:Models, Biological; D009154:Mutation; D012323:RNA Processing, Post-Transcriptional; D012337:RNA, Ribosomal, 18S / Q000235:genetics* / Q000378:metabolism*; D012339:RNA, Ribosomal, 28S / Q000235:genetics / Q000378:metabolism; D034741:RNA, Small Interfering / Q000235:genetics; D012269:Ribosomal Proteins / Q000037:antagonists & inhibitors / Q000737:chemistry / Q000172:deficiency / Q000235:genetics* / Q000378:metabolism*
chemical_list: D012337:RNA, Ribosomal, 18S; D012339:RNA, Ribosomal, 28S; D034741:RNA, Small Interfering; D012269:Ribosomal Proteins; C040343:ribosomal protein S19