Ribosomes and marrow failure: coincidental association or molecular paradigm?
about
Human RPS19, the gene mutated in Diamond-Blackfan anemia, encodes a ribosomal protein required for the maturation of 40S ribosomal subunitsThe human Shwachman-Diamond syndrome protein, SBDS, associates with ribosomal RNAIdentification of RPS14 as a 5q- syndrome gene by RNA interference screenImpaired ribosome biogenesis in Diamond-Blackfan anemiaSBDS expression and localization at the mitotic spindle in human myeloid progenitorsAn RNA-dependent RNA polymerase formed by TERT and the RMRP RNAAbnormalities of the large ribosomal subunit protein, Rpl35a, in Diamond-Blackfan anemiaEvolutionary conservation and expression of human RNA-binding proteins and their role in human genetic diseaseThe inherited bone marrow failure syndromesGuarding the 'translation apparatus': defective ribosome biogenesis and the p53 signaling pathwayEukaryotic translation initiation factor 6 is a novel regulator of reactive oxygen species-dependent megakaryocyte maturationChanges in rRNA transcription influence proliferation and cell fate within a stem cell lineage.The ribosomal protein Rpl22 controls ribosome composition by directly repressing expression of its own paralog, Rpl22l1Ribosomal protein gene knockdown causes developmental defects in zebrafish.Fibroblasts from patients with Diamond-Blackfan anaemia show abnormal expression of genes involved in protein synthesis, amino acid metabolism and cancer.The human RPS4 paralogue on Yq11.223 encodes a structurally conserved ribosomal protein and is preferentially expressed during spermatogenesis.Distinct ribosome maturation defects in yeast models of Diamond-Blackfan anemia and Shwachman-Diamond syndromeThe dichotomy of p53 regulation by noncoding RNAs.Ribosomopathies: human disorders of ribosome dysfunction.Ribosomal protein S19 binds to its own mRNA with reduced affinity in Diamond-Blackfan anemiaDiamond Blackfan anemia 2008-2009: broadening the scope of ribosome biogenesis disorders.Diamond-Blackfan anemia: diagnosis, treatment, and molecular pathogenesis.Effect of HIP/ribosomal protein L29 deficiency on mineral properties of murine bones and teeth.Functional dichotomy of ribosomal proteins during the synthesis of mammalian 40S ribosomal subunits.Non-Diamond Blackfan anemia disorders of ribosome function: Shwachman Diamond syndrome and 5q- syndromeRibosomal proteins and human diseases: pathogenesis, molecular mechanisms, and therapeutic implicationsHaploinsufficiency for ribosomal protein genes causes selective activation of p53 in human erythroid progenitor cells.Mutation of a gene essential for ribosome biogenesis, EMG1, causes Bowen-Conradi syndrome.Exploiting pre-rRNA processing in Diamond Blackfan anemia gene discovery and diagnosisSignaling to p53: ribosomal proteins find their way.In Vivo Senescence in the Sbds-Deficient Murine Pancreas: Cell-Type Specific Consequences of Translation Insufficiency.Ribosomal L22-like1 (RPL22L1) Promotes Ovarian Cancer Metastasis by Inducing Epithelial-to-Mesenchymal TransitionMutation of kri1l causes definitive hematopoiesis failure via PERK-dependent excessive autophagy induction.Biology of the bone marrow microenvironment and myelodysplastic syndromes.Telomere maintenance and human bone marrow failureAlopecia, neurological defects, and endocrinopathy syndrome caused by decreased expression of RBM28, a nucleolar protein associated with ribosome biogenesisSome cases of common variable immunodeficiency may be due to a mutation in the SBDS gene of Shwachman-Diamond syndrome.Ribosomal protein L29/HIP deficiency delays osteogenesis and increases fragility of adult bone in mice.Deletion 5q in myelodysplastic syndrome: a paradigm for the study of hemizygous deletions in cancer.RPL27A is a target of miR-595 and may contribute to the myelodysplastic phenotype through ribosomal dysgenesis
P2860
Q24304155-D61B6A8E-9A83-4976-87A1-599199142A4DQ24304508-45D79DE9-EDE9-4EAA-AC1D-F4634FE53F80Q24306795-1643574A-455C-4130-9D18-F2DD28CB2A39Q24307762-E8682E2B-36C8-4E5C-975E-7A00ACF49950Q24316999-D7583341-171A-46EE-ABF3-0B979B4A801CQ24336971-17FDC1E8-D716-41FD-A283-44E32AB179A2Q24642192-ACB1297E-8E40-4DF1-AA05-BCAF3670F5ACQ26991754-8F4BE04F-EB35-46F4-B4CC-468914AA72DBQ26999046-68DF5C95-0171-4471-B239-FC56474F5A43Q27022918-D4BAA438-DE64-47EB-927B-11DEA4E18797Q28506261-28A0C5CA-6EA5-45BF-A57C-02B0DD1EA3C4Q30583079-FCEDC6AA-5F27-4E0D-8750-6433D51D52B6Q31129965-828132AF-4046-4C99-9A37-D5C78900FD81Q33267296-1D9749DB-7656-4EBB-BF8C-097A7257E089Q33504868-7D3DF8C7-4977-44C1-A7AD-A4FB32144A64Q33575168-EB29A2A5-7E6F-4148-8D3D-6B87AE64A126Q33587612-0850EF8D-A6B1-4D05-931C-25F95667C596Q33668469-C4CD9691-1FAD-4F26-93F8-DBF42A96B95BQ33806050-383D6344-375E-4CF4-939B-8AFC95156CE1Q33886441-963F0A3C-576E-42A2-8489-F27909DA571AQ33916824-2A76B9D9-8B9A-4A70-A4D1-DA2267ED48A4Q33916840-CCCC0BCF-EA2A-4FC1-866C-49B846FB4D46Q33941292-8C525360-BA9F-40B8-849D-CB293C6E3E98Q34110999-EA1FA304-5C47-43A6-AEAF-9D7758FE0BD2Q34173140-A23DE9F1-9F46-4EF8-ADE3-912A070974CFQ34435519-BC040BE5-897C-4860-91D5-A28122D606DAQ34707415-45A6984E-DC86-4772-A9D1-0F738ADAD9FEQ34982627-1084E0B1-51CB-47FD-B1E1-113712EB0353Q35095302-394CBCA2-CFC6-47D8-BE77-419473C33846Q35206381-9FCCEF6B-DD23-4D2D-9F9E-8F365B2B3D9EQ35658181-B31E816C-D02D-4C11-93A0-0B405B558FA7Q35855606-64EFD65B-218D-42AE-ADB2-96A1FCB0C4DDQ35925596-4BAC4ED1-D787-43F4-B972-1880006F62A9Q36201068-7559B5C1-169E-42E6-AF11-39A0A0CC3E30Q36591289-940A8407-7667-47B4-920A-DDA026B1F5B6Q36719169-3D55640B-CFBC-430F-8A50-0E0B8D65C528Q37055288-FD81EA57-BC41-4BAB-A291-5D35D8D47B3DQ37101839-1347ED43-2307-4EE9-B353-543D1AF59B58Q37423777-E98E737B-C79D-4337-B3CA-35FBD03915ABQ37565308-BF084E13-C3B6-4C25-862B-6A45B6CCDA85
P2860
Ribosomes and marrow failure: coincidental association or molecular paradigm?
description
2006 nî lūn-bûn
@nan
2006年の論文
@ja
2006年学术文章
@wuu
2006年学术文章
@zh-cn
2006年学术文章
@zh-hans
2006年学术文章
@zh-my
2006年学术文章
@zh-sg
2006年學術文章
@yue
2006年學術文章
@zh
2006年學術文章
@zh-hant
name
Ribosomes and marrow failure: coincidental association or molecular paradigm?
@ast
Ribosomes and marrow failure: coincidental association or molecular paradigm?
@en
type
label
Ribosomes and marrow failure: coincidental association or molecular paradigm?
@ast
Ribosomes and marrow failure: coincidental association or molecular paradigm?
@en
prefLabel
Ribosomes and marrow failure: coincidental association or molecular paradigm?
@ast
Ribosomes and marrow failure: coincidental association or molecular paradigm?
@en
P1433
P1476
Ribosomes and marrow failure: coincidental association or molecular paradigm?
@en
P2093
Johnson M Liu
Steven R Ellis
P304
P356
10.1182/BLOOD-2005-12-4831
P407
P577
2006-02-28T00:00:00Z