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10.1016/j.jbior.2017.09.002 http://scihub22266oqcxt.onion/10.1016/j.jbior.2017.09.002 28942353MolecularbasisofthehumanribosomopathyShwachman-Diamondsyndrome.!6710477!28942353     free     free     free Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       Adv+Biol+Regul 2018 ; 67 (ä): 109-127 Nephropedia Template TP {{tp|p=28942353|t=2018. Molecular basis of the human ribosomopathy Shwachman-Diamond syndrome |pdf=|usr=}}{{28942353}} gab.com Text Molecular basis of the human ribosomopathy Shwachman-Diamond syndrome JO: Adv Biol Regul http://www.kidney.de/pget.php?&tw=1&pmid=28942353 #FOAvip Mutations that target the ubiquitous process of ribosome assembly paradoxically cause diverse tissue-specific disorders (ribosomopathies) that are often associated with an increased risk of cancer. Ribosomes are the essential macromolecular machines that read the genetic code in all cells in all kingdoms of life. Following pre-assembly in the nucleus, precursors of the large 60S and small 40S ribosomal subunits are exported to the cytoplasm where the final steps in maturation are completed. Here, I review the recent insights into the conserved mechanisms of ribosome assembly that have come from functional characterisation of the genes mutated in human ribosomopathies. In particular, recent advances in cryo-electron microscopy, coupled with genetic, biochemical and prior structural data, have revealed that the SBDS protein that is deficient in the inherited leukaemia predisposition disorder Shwachman-Diamond syndrome couples the final step in cytoplasmic 60S ribosomal subunit maturation to a quality control assessment of the structural and functional integrity of the nascent particle. Thus, study of this fascinating disorder is providing remarkable insights into how the large ribosomal subunit is functionally activated in the cytoplasm to enter the actively translating pool of ribosomes. Twit Text FOAVip Molecular basis of the human ribosomopathy Shwachman-Diamond syndrome ????Adv Biol Regul http://www.kidney.de/pget.php?&tw=1&pmid=28942353 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=28942353 #FOAvip English Wikipedia <ref>{{Cite pmid|28942353}}</ref> Molecular basis of the human ribosomopathy Shwachman-Diamond syndrome #MMPMID28942353 Warren AJ Adv Biol Regul 2018[Jan]; 67 (ä): 109-127 PMID28942353show ga Mutations that target the ubiquitous process of ribosome assembly paradoxically cause diverse tissue-specific disorders (ribosomopathies) that are often associated with an increased risk of cancer. Ribosomes are the essential macromolecular machines that read the genetic code in all cells in all kingdoms of life. Following pre-assembly in the nucleus, precursors of the large 60S and small 40S ribosomal subunits are exported to the cytoplasm where the final steps in maturation are completed. Here, I review the recent insights into the conserved mechanisms of ribosome assembly that have come from functional characterisation of the genes mutated in human ribosomopathies. In particular, recent advances in cryo-electron microscopy, coupled with genetic, biochemical and prior structural data, have revealed that the SBDS protein that is deficient in the inherited leukaemia predisposition disorder Shwachman-Diamond syndrome couples the final step in cytoplasmic 60S ribosomal subunit maturation to a quality control assessment of the structural and functional integrity of the nascent particle. Thus, study of this fascinating disorder is providing remarkable insights into how the large ribosomal subunit is functionally activated in the cytoplasm to enter the actively translating pool of ribosomes. |*Mutation[MESH] |Bone Marrow Diseases/*metabolism/pathology[MESH] |Cryoelectron Microscopy[MESH] |Exocrine Pancreatic Insufficiency/*metabolism/pathology[MESH] |Humans[MESH] |Lipomatosis/*metabolism/pathology[MESH] |Proteins/genetics/*metabolism[MESH] |Ribosome Subunits, Large, Eukaryotic/genetics/*metabolism/ultrastructure[MESH] |Ribosome Subunits, Small, Eukaryotic/genetics/metabolism/ultrastructure[MESH]Molecular basis of the human ribosomopathy Shwachman-Diamond syndrome (epmc).pdf DeepDyve Pubget Overpricing