Myelofibrosis with myeloid metaplasia (MMM) is a clonal disorder involving disregulation of angiogenesis and immunomodulatory mechanisms. Thalidomide (Thal) retains antiangiogenic, immunomodulatory and cytokine regulatory properties and recently it has been used successfully in multiple myeloma. Here, we report our experience in 10 MMM patients treated with Thal. Patients with agnogenic MMM treated in an early phase of the disease obtained significant benefits from the therapy and remain transfusion-free. In contrast, all secondary MMM failed to respond. These preliminary findings confirm that Thal plays a role in MMM therapy, although the efficacy in the different phases of the disease must be further evaluated.
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http://dx.doi.org/10.1046/j.1365-2141.2001.03072.x | DOI Listing |
Nat Rev Mol Cell Biol
January 2025
Donnelly Centre for Cellular and Biomolecular Research and Department of Biochemistry, Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.
In multicellular organisms, Wnt proteins govern stem and progenitor cell renewal and differentiation to regulate embryonic development, adult tissue homeostasis and tissue regeneration. Defects in canonical Wnt signalling, which is transduced intracellularly by β-catenin, have been associated with developmental disorders, degenerative diseases and cancers. Although a simple model describing Wnt-β-catenin signalling is widely used to introduce this pathway and has largely remained unchanged over the past 30 years, in this Review we discuss recent studies that have provided important new insights into the mechanisms of Wnt production, receptor activation and intracellular signalling that advance our understanding of the molecular mechanisms that underlie this important cell-cell communication system.
View Article and Find Full Text PDFMembranes (Basel)
January 2025
State Key Laboratory of Materials-Oriented Chemical Engineering, College of Chemical Engineering, Nanjing Tech University, Nanjing 210009, China.
Membrane technology is a promising methodology for carbon dioxide separation due to its benefit of a small carbon footprint. However, the trade-off relationship between gas permeability and selectivity is one obstacle to limiting its application. Herein, branched polyethyleneimine (BPEI) containing a rich amino group was successfully grafted on the surface of the metal-organic framework (MOF) of AIFFIVE-1-Ni (KAUST-8) through coordination between N in BPEI and open metal sites in the MOF and with the resultant maintained BET surface area and pore volume.
View Article and Find Full Text PDFAJNR Am J Neuroradiol
January 2025
Ataxia Center, Cognitive Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology (J.D.S., Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts.
Background And Purpose: Symptoms indistinguishable from behavioral-variant frontotemporal dementia (bvFTD) can develop in patients with spontaneous intracranial hypotension associated with severe brain sagging. An underlying spinal CSF leak can be identified in only a minority of these patients and the success rate of nondirected treatments, such as epidural blood patching and dural reduction surgery, is low. The disability associated with bvFTD sagging brain syndrome is high and, because of the importance of the venous system in the pathophysiology of CSF leaks in general, we have investigated the systemic venous circulation in those patients with recalcitrant symptoms.
View Article and Find Full Text PDFSci Rep
January 2025
Wollega University, Nekemte, Ethiopia.
This research paper presents an advanced AI-driven hybrid power quality management system for electrical railways that addresses critical challenges in 25 kV AC traction networks through a novel integration of single-phase PV-UPQC with ANN-Lyapunov control architecture. The system effectively manages voltage unbalance exceeding 2%, high THD, voltage variations of ± 10%, and poor power factor through a dual-approach methodology combining ANN-based reference signal generation with Lyapunov optimization, enabling dynamic parameter tuning and real-time load adaptation. MATLAB/Simulink simulations validate the system's superior performance, demonstrating significant improvements, including voltage unbalance reduction from 1.
View Article and Find Full Text PDFAppl Radiat Isot
January 2025
Radiopharmaceuticals Division, Bhabha Atomic Research Centre, Trombay, Mumbai, 400085, India.
F radioactive isotope is widely used in PET imaging for nuclear medicine. Medical linear accelerators producing high-flux bremsstrahlung beams up to 20 MeV are commonly used in radiation therapy. Hence, the production of F through photon-induced channels will reduce many of the intricacies in transportation and handling.
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