Journal
BRITISH JOURNAL OF HAEMATOLOGY
Volume 141, Issue 3, Pages 376-387Publisher
WILEY
DOI: 10.1111/j.1365-2141.2008.07095.x
Keywords
marrow failure; haematopoiesis; ribosome; protein translation; cancer
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Impairment of ribosome biogenesis or function characterizes several of the inherited bone marrow failure syndromes: Diamond-Blackfan anaemia, dyskeratosis congenita (DC), Shwachman-Diamond syndrome and cartilage-hair hypoplasia. These syndromes exhibit overlapping but distinct clinical phenotypes and each disorder involves different aspects of ribosomal biogenesis. The clinical characteristics of each syndrome are briefly reviewed. Molecular studies of ribosome biogenesis and function in each of these syndromes are discussed. Models of how impairment of ribosomal pathways might affect haematopoiesis and tumorigenesis are explored.
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