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Mesenchymal Inflammation Drives Genotoxic Stress in Hematopoietic Stem Cells and Predicts Disease Evolution in Human Pre-leukemia.
Mesenchymal niche cells may drive tissue failure and malignant transformation in the hematopoietic system, but the underlying molecular mechanisms and relevance to human disease remain poorlyExpand
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Deficiency of the ribosome biogenesis gene Sbds in hematopoietic stem and progenitor cells causes neutropenia in mice by attenuating lineage progression in myelocytes
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,Expand
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‘Selectin’ endothelium to protect blood stem cells
Hematopoietic stem cells (HSC) reside in anatomically defined regions that control their number and fate. These are referred to as the bone marrow niche or microenvironment. The composition of thisExpand
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Inflammatory Niche Signalling Drives Genotoxic Stress in Hematopoietic Stem Cells and Predicts Leukemic Evolution in Human Leukemia Predisposition Syndromes
Primary alterations of the mesenchymal niche can induce myelodysplasia and acute myeloid leukemia in mouse models, introducing a concept of niche-driven leukemogenesis (Raaijmakers et al, NatureExpand
Abstract A25: In vivo evidence validating the palmitoylation/depalmitoylation cycle as a therapeutic target in NRAS mutant hematologic cancers
A dynamic cycle of palmitoylation and depalmitoylation stably localizes N-Ras, but not K-Ras-4b, at the plasma membrane. This selective dependency suggests that targeting palmitoylation could inhibitExpand
Deletion of Sbds from Hematopoietic Progenitors Causes Neutropenia in a Mouse Model of Shwachman-Diamond Syndrome By Specifically Blocking Myeloid Lineage Progression at Late Differentiation Stages
Shwachman-Diamond Syndrome (SDS) is a congenital bone marrow failure disorder characterized by neutropenia and predisposition to leukemia. SDS is associated with loss-of-function mutations in theExpand
Oncogenic niche signaling in human leukemia predisposition syndromes
Mesenchymal niche cells may drive tissue failure and malignant transformation in the hematopoietic system but the molecular mechanisms and their relevance to human disease remain poorly defined.Expand
ABHD17 enzymes regulate dynamic plasma membrane palmitoylation and N-Ras-dependent cancer growth
A subset of Ras proteins, including N-Ras, depend on a palmitoylation/depalmitoylation cycle to regulate their subcellular trafficking and oncogenicity. General lipase inhibitors such as PalmostatinExpand
Deconstructing Niche Contributions to Leukemogenesis: Modeling Shwachman-Diamond Syndrome
markdownabstractHematopoietic stem and progenitor cells (HSPC) proliferate and differentiate to provide a lifelong production of blood cells, but sustained proliferation puts them at risk ofExpand