Literature DB >> 19454283

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

Jitendra Badhai1, Anne-Sophie Fröjmark, Hamid Reza Razzaghian, Edward Davey, Jens Schuster, Niklas Dahl.   

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.

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Year:  2009        PMID: 19454283      PMCID: PMC2737615          DOI: 10.1016/j.febslet.2009.05.023

Source DB:  PubMed          Journal:  FEBS Lett        ISSN: 0014-5793            Impact factor:   4.124


  25 in total

1.  Transcriptional elements involved in the repression of ribosomal protein synthesis.

Authors:  B Li; C R Nierras; J R Warner
Journal:  Mol Cell Biol       Date:  1999-08       Impact factor: 4.272

Review 2.  The economics of ribosome biosynthesis in yeast.

Authors:  J R Warner
Journal:  Trends Biochem Sci       Date:  1999-11       Impact factor: 13.807

3.  Diamond-Blackfan anaemia: genetic homogeneity for a gene on chromosome 19q13 restricted to 1.8 Mb.

Authors:  P Gustavsson; T N Willing; A van Haeringen; G Tchernia; I Dianzani; M Donnér; G Elinder; J I Henter; P G Nilsson; L Gordon; G Skeppner; L van't Veer-Korthof; A Kreuger; N Dahl
Journal:  Nat Genet       Date:  1997-08       Impact factor: 38.330

4.  Development of cellular models for ribosomal protein S19 (RPS19)-deficient diamond-blackfan anemia using inducible expression of siRNA against RPS19.

Authors:  Koichi Miyake; Johan Flygare; Thomas Kiefer; Taiju Utsugisawa; Johan Richter; Zhi Ma; Maciej Wiznerowicz; Didier Trono; Stefan Karlsson
Journal:  Mol Ther       Date:  2005-04       Impact factor: 11.454

5.  A human protein atlas for normal and cancer tissues based on antibody proteomics.

Authors:  Mathias Uhlén; Erik Björling; Charlotta Agaton; Cristina Al-Khalili Szigyarto; Bahram Amini; Elisabet Andersen; Ann-Catrin Andersson; Pia Angelidou; Anna Asplund; Caroline Asplund; Lisa Berglund; Kristina Bergström; Harry Brumer; Dijana Cerjan; Marica Ekström; Adila Elobeid; Cecilia Eriksson; Linn Fagerberg; Ronny Falk; Jenny Fall; Mattias Forsberg; Marcus Gry Björklund; Kristoffer Gumbel; Asif Halimi; Inga Hallin; Carl Hamsten; Marianne Hansson; My Hedhammar; Görel Hercules; Caroline Kampf; Karin Larsson; Mats Lindskog; Wald Lodewyckx; Jan Lund; Joakim Lundeberg; Kristina Magnusson; Erik Malm; Peter Nilsson; Jenny Odling; Per Oksvold; Ingmarie Olsson; Emma Oster; Jenny Ottosson; Linda Paavilainen; Anja Persson; Rebecca Rimini; Johan Rockberg; Marcus Runeson; Asa Sivertsson; Anna Sköllermo; Johanna Steen; Maria Stenvall; Fredrik Sterky; Sara Strömberg; Mårten Sundberg; Hanna Tegel; Samuel Tourle; Eva Wahlund; Annelie Waldén; Jinghong Wan; Henrik Wernérus; Joakim Westberg; Kenneth Wester; Ulla Wrethagen; Lan Lan Xu; Sophia Hober; Fredrik Pontén
Journal:  Mol Cell Proteomics       Date:  2005-08-27       Impact factor: 5.911

6.  Deficiency of ribosomal protein S19 in CD34+ cells generated by siRNA blocks erythroid development and mimics defects seen in Diamond-Blackfan anemia.

Authors:  Johan Flygare; Thomas Kiefer; Koichi Miyake; Taiju Utsugisawa; Isao Hamaguchi; Lydie Da Costa; Johan Richter; Edward J Davey; Hans Matsson; Niklas Dahl; Maciej Wiznerowicz; Didier Trono; Stefan Karlsson
Journal:  Blood       Date:  2004-12-30       Impact factor: 22.113

7.  An RNA interference model of RPS19 deficiency in Diamond-Blackfan anemia recapitulates defective hematopoiesis and rescue by dexamethasone: identification of dexamethasone-responsive genes by microarray.

Authors:  Benjamin L Ebert; Michele M Lee; Jennifer L Pretz; Aravind Subramanian; Raymond Mak; Todd R Golub; Colin A Sieff
Journal:  Blood       Date:  2005-03-08       Impact factor: 22.113

8.  RNA and protein evidence for haplo-insufficiency in Diamond-Blackfan anaemia patients with RPS19 mutations.

Authors:  Hanna T Gazda; Rong Zhong; Lilia Long; Edyta Niewiadomska; Jeffrey M Lipton; Anna Ploszynska; Jan M Zaucha; Adrianna Vlachos; Evangelia Atsidaftos; David H Viskochil; Charlotte M Niemeyer; Joerg J Meerpohl; Roma Rokicka-Milewska; Dagmar Pospisilova; W Wiktor-Jedrzejczak; David G Nathan; Alan H Beggs; Colin A Sieff
Journal:  Br J Haematol       Date:  2004-10       Impact factor: 6.998

9.  Ribosomal protein L5 and L11 mutations are associated with cleft palate and abnormal thumbs in Diamond-Blackfan anemia patients.

Authors:  Hanna T Gazda; Mee Rie Sheen; Adrianna Vlachos; Valerie Choesmel; Marie-Françoise O'Donohue; Hal Schneider; Natasha Darras; Catherine Hasman; Colin A Sieff; Peter E Newburger; Sarah E Ball; Edyta Niewiadomska; Michal Matysiak; Jan M Zaucha; Bertil Glader; Charlotte Niemeyer; Joerg J Meerpohl; Eva Atsidaftos; Jeffrey M Lipton; Pierre-Emmanuel Gleizes; Alan H Beggs
Journal:  Am J Hum Genet       Date:  2008-12       Impact factor: 11.025

10.  The gene encoding ribosomal protein S19 is mutated in Diamond-Blackfan anaemia.

Authors:  N Draptchinskaia; P Gustavsson; B Andersson; M Pettersson; T N Willig; I Dianzani; S Ball; G Tchernia; J Klar; H Matsson; D Tentler; N Mohandas; B Carlsson; N Dahl
Journal:  Nat Genet       Date:  1999-02       Impact factor: 38.330

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  14 in total

1.  The effects of EBV transformation on gene expression levels and methylation profiles.

Authors:  Minal Caliskan; Darren A Cusanovich; Carole Ober; Yoav Gilad
Journal:  Hum Mol Genet       Date:  2011-02-02       Impact factor: 6.150

2.  Ribosomal protein S19 binds to its own mRNA with reduced affinity in Diamond-Blackfan anemia.

Authors:  Jens Schuster; Anne-Sophie Fröjmark; Per Nilsson; Jitendra Badhai; Anders Virtanen; Niklas Dahl
Journal:  Blood Cells Mol Dis       Date:  2010-04-14       Impact factor: 3.039

3.  A single-nucleotide deletion in the POMP 5' UTR causes a transcriptional switch and altered epidermal proteasome distribution in KLICK genodermatosis.

Authors:  Johanna Dahlqvist; Joakim Klar; Neha Tiwari; Jens Schuster; Hans Törmä; Jitendra Badhai; Ramon Pujol; Maurice A M van Steensel; Tjinta Brinkhuizen; Tjinta Brinkhuijzen; Lieke Gijezen; Antonio Chaves; Gianluca Tadini; Anders Vahlquist; Niklas Dahl
Journal:  Am J Hum Genet       Date:  2010-03-11       Impact factor: 11.025

4.  Mutations in RPS19 may affect ribosome function and biogenesis in Diamond Blackfan anemia.

Authors:  Disha-Gajanan Hiregange; Andre Rivalta; Ada Yonath; Ella Zimmerman; Anat Bashan; Hagith Yonath
Journal:  FEBS Open Bio       Date:  2022-06-06       Impact factor: 2.792

5.  Elevation of receptor tyrosine kinases by small molecule AKT inhibitors in prostate cancer is mediated by Pim-1.

Authors:  Bo Cen; Sandeep Mahajan; Wenxue Wang; Andrew S Kraft
Journal:  Cancer Res       Date:  2013-04-12       Impact factor: 12.701

6.  Silencing of ribosomal protein S9 elicits a multitude of cellular responses inhibiting the growth of cancer cells subsequent to p53 activation.

Authors:  Mikael S Lindström; Monica Nistér
Journal:  PLoS One       Date:  2010-03-08       Impact factor: 3.240

7.  Diamond Blackfan Anemia at the Crossroad between Ribosome Biogenesis and Heme Metabolism.

Authors:  Deborah Chiabrando; Emanuela Tolosano
Journal:  Adv Hematol       Date:  2010-05-05

8.  Ribosomal protein S19 and S24 insufficiency cause distinct cell cycle defects in Diamond-Blackfan anemia.

Authors:  Jitendra Badhai; Anne-Sophie Fröjmark; Edward J Davey; Jens Schuster; Niklas Dahl
Journal:  Biochim Biophys Acta       Date:  2009-08-16

Review 9.  RNA binding protein/RNA element interactions and the control of translation.

Authors:  Xavier Pichon; Lindsay A Wilson; Mark Stoneley; Amandine Bastide; Helen A King; Joanna Somers; Anne E E Willis
Journal:  Curr Protein Pept Sci       Date:  2012-06       Impact factor: 3.272

10.  siRNA silencing of proteasome maturation protein (POMP) activates the unfolded protein response and constitutes a model for KLICK genodermatosis.

Authors:  Johanna Dahlqvist; Hans Törmä; Jitendra Badhai; Niklas Dahl
Journal:  PLoS One       Date:  2012-01-03       Impact factor: 3.240

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