ArticleHaematologica2015
Deficiency of the ribosome biogenesis gene Sbds in hematopoietic stem and progenitor cells causes neutropenia in mice by attenuating lineage progression in myelocytes.
Article in Haematologica, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers.
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Who cites it
34 citing papers in PubMed, 53 citations in OpenAlex.
- A Multi-omics and Machine Learning Framework Identifies Plasma SBDS as a Causal Biomarker and Therapeutic Target in Primary Sclerosing Cholangitis.Journal of clinical and translational hepatology · 2026Article
- Analyzing the role of ferroptosis in ribosome-related bone marrow failure disorders: From pathophysiology to potential pharmacological exploitation.IUBMB life · 2024Review
- Knockdown of the Shwachman-Diamond syndrome gene, SBDS, induces galectin-1 expression and impairs cell growth.International journal of hematology · 2024Article
- Shwachman-Diamond syndromes: clinical, genetic, and biochemical insights from the rare variants.Haematologica · 2023Article
- Convergent somatic evolution commences in utero in a germline ribosomopathy.Nature communications · 2023Article
- Hematopoietic Cell Autonomous Disruption of Hematopoiesis in a Germline Loss-of-function Mouse Model ofHemaSphere · 2023Article
- FCN3 inhibits the progression of hepatocellular carcinoma by suppressing SBDS-mediated blockade of the p53 pathway.International journal of biological sciences · 2023Article
- Myeloid cells promote interferon signaling-associated deterioration of the hematopoietic system.Nature communications · 2022Article
- Genetics of severe congenital neutropenia as a gateway to personalized therapy.Hematology. American Society of Hematology. Education Program · 2022Article
- Induced cell-autonomous neutropenia systemically perturbs hematopoiesis in Cebpa enhancer-null mice.Blood advances · 2022Article
- Inducible Sbds deletion impairs bone marrow niche capacity to engraft donor bone marrow after transplantation.Blood advances · 2022Article
- Impaired myelopoiesis in congenital neutropenia: insights into clonal and malignant hematopoiesis.Hematology. American Society of Hematology. Education Program · 2021Article
- An integrative proteomics method identifies a regulator of translation during stem cell maintenance and differentiation.Nature communications · 2021Article
- Translational control of stem cell function.Nature reviews. Molecular cell biology · 2021Review
- Article
- Pluripotent stem cell model of Shwachman-Diamond syndrome reveals apoptotic predisposition of hemoangiogenic progenitors.Scientific reports · 2020Article
- On-chip recapitulation of clinical bone marrow toxicities and patient-specific pathophysiology.Nature biomedical engineering · 2020Article
- Mesenchymal Stromal Cells as a Cellular Target in Myeloid Malignancy: Chances and Challenges in the Genome Editing of Stromal Alterations.Frontiers in genome editing · 2020Review
- TGFβ signaling underlies hematopoietic dysfunction and bone marrow failure in Shwachman-Diamond Syndrome.The Journal of clinical investigation · 2019Article
- Peripheral blood immunophenotyping in a large cohort of patients with Shwachman-Diamond syndrome.Pediatric blood & cancer · 2019Article
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Authors and funding
10 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Shwachman-Diamond syndrome is a congenital bone marrow failure disorder characterized by debilitating neutropenia. The disease is associated with loss-of-function mutations in the SBDS gene, implicated in ribosome biogenesis, but the cellular and molecular events driving cell specific phenotypes in ribosomopathies remain poorly defined. Here, we established what is to our knowledge the first mammalian model of neutropenia in Shwachman-Diamond syndrome through targeted downregulation of Sbds in hematopoietic stem and progenitor cells expressing the myeloid transcription factor CCAAT/enhancer binding protein α (Cebpa). Sbds deficiency in the myeloid lineage specifically affected myelocytes and their downstream progeny while, unexpectedly, it was well tolerated by rapidly cycling hematopoietic progenitor cells. Molecular insights provided by massive parallel sequencing supported cellular observations of impaired cell cycle exit and formation of secondary granules associated with the defect of myeloid lineage progression in myelocytes. Mechanistically, Sbds deficiency activated the p53 tumor suppressor pathway and induced apoptosis in these cells. Collectively, the data reveal a previously unanticipated, selective dependency of myelocytes and downstream progeny, but not rapidly cycling progenitors, on this ubiquitous ribosome biogenesis protein, thus providing a cellular basis for the understanding of myeloid lineage biased defects in Shwachman-Diamond syndrome.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.