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Blood, 15 June 2006, Vol. 107, No. 12, pp. 4583-4588. Prepublished online as a Blood First Edition Paper on February 28, 2006; DOI 10.1182/blood-2005-12-4831. This Article Full Text (PDF) All Versions of this Article: 2005-12-4831v1 107/12/4583    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Liu, J. M Articles by Ellis, S. R Search for Related Content PubMed PubMed Citation Articles by Liu, J. M Articles by Ellis, S. R Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted December 6, 2005 Accepted February 9, 2006 Ribosomes and marrow failure: coincidental association or molecular paradigm? Johnson M Liu* and Steven R Ellis Feinstein Institute for Medical Research, Manhasset, NY, USA; Schneider Children's Hospital, New Hyde Park, NY, USA Department of Biochemistry and Molecular Biology, University of Louisville School of Medicine, Louisville, KY, USA * Corresponding author; email: JLiu3{at}NSHS.edu. Gene products mutated in the inherited bone marrow failure syndromes dyskeratosis congenita (DC), cartilage-hair hypoplasia (CHH), Diamond-Blackfan anemia (DBA), and Shwachman-Diamond syndrome (SDS), are all predicted to be involved in different aspects of ribosome synthesis. At this moment, however, it is unclear whether this link indicates a causal relationship. Although defective ribosome synthesis may contribute to each of these bone marrow failure syndromes (and perhaps others), precisely which feature of each disease is a consequence of failure to produce adequate amounts of ribosomes is obscured by the tendency of each gene product to have extraribosomal functions. Delineation of the precise role of each gene product in ribosomal biogenesis and in hematopoietic development may have both therapeutic and prognostic importance and perhaps even direct the search for new bone marrow failure genes. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: N. Danilova, K. M. Sakamoto, and S. Lin Ribosomal protein S19 deficiency in zebrafish leads to developmental abnormalities and defective erythropoiesis through activation of p53 protein family Blood, December 15, 2008; 112(13): 5228 - 5237. [Abstract] [Full Text] [PDF] I. Dianzani and F. Loreni Diamond-Blackfan anemia: a ribosomal puzzle Haematologica, November 1, 2008; 93(11): 1601 - 1604. [Full Text] [PDF] A. Cretien, C. Hurtaud, H. Moniz, A. Proust, I. Marie, O. Wagner-Ballon, V. Choesmel, P.-E. Gleizes, T. Leblanc, J. Delaunay, et al. Study of the effects of proteasome inhibitors on ribosomal protein S19 (RPS19) mutants, identified in patients with Diamond-Blackfan anemia Haematologica, November 1, 2008; 93(11): 1627 - 1634. [Abstract] [Full Text] [PDF] T. Uechi, Y. Nakajima, A. Chakraborty, H. Torihara, S. Higa, and N. Kenmochi Deficiency of ribosomal protein S19 during early embryogenesis leads to reduction of erythrocytes in a zebrafish model of Diamond-Blackfan anemia Hum. Mol. Genet., October 15, 2008; 17(20): 3204 - 3211. [Abstract] [Full Text] [PDF] J. E. Farrar, M. Nater, E. Caywood, M. A. McDevitt, J. Kowalski, C. M. Takemoto, C. C. Talbot Jr, P. Meltzer, D. Esposito, A. H. Beggs, et al. Abnormalities of the large ribosomal subunit protein, Rpl35a, in Diamond-Blackfan anemia Blood, September 1, 2008; 112(5): 1582 - 1592. [Abstract] [Full Text] [PDF] V. Choesmel, S. Fribourg, A.-H. Aguissa-Toure, N. Pinaud, P. Legrand, H. T. Gazda, and P.-E. Gleizes Mutation of ribosomal protein RPS24 in Diamond-Blackfan anemia results in a ribosome biogenesis disorder Hum. Mol. Genet., May 1, 2008; 17(9): 1253 - 1263. [Abstract] [Full Text] [PDF] R. T. Calado and N. S. Young Telomere maintenance and human bone marrow failure Blood, May 1, 2008; 111(9): 4446 - 4455. [Abstract] [Full Text] [PDF] S. Karlsson Connecting ribosomes with bone marrow failure Blood, September 1, 2007; 110(5): 1402 - 1402. [Full Text] [PDF] K. A. Ganapathi, K. M. Austin, C.-S. Lee, A. Dias, M. M. Malsch, R. Reed, and A. Shimamura The human Shwachman-Diamond syndrome protein, SBDS, associates with ribosomal RNA Blood, September 1, 2007; 110(5): 1458 - 1465. [Abstract] [Full Text] [PDF] M. Angelini, S. Cannata, V. Mercaldo, L. Gibello, C. Santoro, I. Dianzani, and F. Loreni Missense mutations associated with Diamond-Blackfan anemia affect the assembly of ribosomal protein S19 into the ribosome Hum. Mol. Genet., July 15, 2007; 16(14): 1720 - 1727. [Abstract] [Full Text] [PDF] J. Flygare and S. Karlsson Diamond-Blackfan anemia: erythropoiesis lost in translation Blood, April 15, 2007; 109(8): 3152 - 3154. [Abstract] [Full Text] [PDF] S. Orru, A. Aspesi, M. Armiraglio, M. Caterino, F. Loreni, M. Ruoppolo, C. Santoro, and I. Dianzani Analysis of the Ribosomal Protein S19 Interactome Mol. Cell. Proteomics, March 1, 2007; 6(3): 382 - 393. [Abstract] [Full Text] [PDF] J. Flygare, A. Aspesi, J. C. Bailey, K. Miyake, J. M. Caffrey, S. Karlsson, and S. R. Ellis Human RPS19, the gene mutated in Diamond-Blackfan anemia, encodes a ribosomal protein required for the maturation of 40S ribosomal subunits Blood, February 1, 2007; 109(3): 980 - 986. [Abstract] [Full Text] [PDF] V. Choesmel, D. Bacqueville, J. Rouquette, J. Noaillac-Depeyre, S. Fribourg, A. Cretien, T. Leblanc, G. Tchernia, L. Da Costa, and P.-E. Gleizes Impaired ribosome biogenesis in Diamond-Blackfan anemia Blood, February 1, 2007; 109(3): 1275 - 1283. [Abstract] [Full Text] [PDF] I. Dokal Fanconi's anaemia and related bone marrow failure syndromes Br. Med. Bull., October 5, 2006; (2006) ldl007v2. [Abstract] [Full Text] [PDF] A. Shimamura Inherited Bone Marrow Failure Syndromes: Molecular Features Hematology, January 1, 2006; 2006(1): 63 - 71. [Abstract] [Full Text] [PDF]

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