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showing 3 items of 553 documents

Novel Translational Read-through–Inducing Drugs as a Therapeutic Option for Shwachman-Diamond Syndrome

2022

Shwachman-Diamond syndrome (SDS) is one of the most commonly inherited bone marrow failure syndromes (IBMFS). In SDS, bone marrow is hypocellular, with marked neutropenia. Moreover, SDS patients have a high risk of developing myelodysplastic syndrome (MDS), which in turn increases the risk of acute myeloid leukemia (AML) from an early age. Most SDS patients are heterozygous for the c.183-184TA>CT (K62X) SBDS nonsense mutation. Fortunately, a plethora of translational read-through inducing drugs (TRIDs) have been developed and tested for several rare inherited diseases due to nonsense mutations so far. The authors previously demonstrated that ataluren (PTC124) can restore full-length SBDS…

neutropeniaMedicine (miscellaneous)atalurenbone marrow failure syndromes; ataluren; neutropeniabone marrow failure syndromesShwachman-Diamond SyndromeGeneral Biochemistry Genetics and Molecular BiologyBiomedicines
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The "gray zone" of the Ph-negative myeloproliferative neoplasm; proposal of a decision tree useful in the differential diagnosis

2010

ph-negative MPNbone marrow biopsySettore MED/08 - Anatomia Patologica
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Extracellular Vesicle microRNAs Contribute to the Osteogenic Inhibition of Mesenchymal Stem Cells in Multiple Myeloma

2020

Osteolytic bone disease is the major complication associated with the progression of multiple myeloma (MM). Recently, extracellular vesicles (EVs) have emerged as mediators of MM-associated bone disease by inhibiting the osteogenic differentiation of human mesenchymal stem cells (hMSCs). Here, we investigated a correlation between the EV-mediated osteogenic inhibition and MM vesicle content, focusing on miRNAs. By the use of a MicroRNA Card, we identified a pool of miRNAs, highly expressed in EVs, from MM cell line (MM1.S EVs), expression of which was confirmed in EVs from bone marrow (BM) plasma of patients affected by smoldering myeloma (SMM) and MM. Notably,we found that miR-129-5p, whic…

transcription factor sp1.Cancer ResearchBone diseaseosteogenic differentiationexosomeslcsh:RC254-282transcription factor sp1ArticleSettore MED/15 - Malattie Del SangueSettore BIO/13 - Biologia Applicatamedicinemultiple myeloma (MM)ChemistrySettore BIO/16 - Anatomia UmanaMesenchymal stem cellALPLOsteoblastMicroRNAExtracellular vesiclemedicine.diseaselcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogensSettore CHIM/08 - Chimica FarmaceuticaCell biologymicroRNAsExosomemedicine.anatomical_structureOncologyCell cultureAlkaline phosphatasebone diseaseBone marrowextracellular vesicles (EVs)Cancers
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