In this work, experimentally measured characteristics of a kilohertz laser-driven Cu plasma X-ray source that was recently commissioned at the ELI Beamlines facility are reported. The source can be driven either by an in-house developed high-contrast sub-20 fs near-infrared terawatt laser based on optical parametric chirped-pulse amplification technology or by a more conventional Ti:sapphire laser delivering 12 mJ and 45 fs pulses. The X-ray source parameters obtained with the two driving lasers are compared. A measured photon flux of the order up to 1012 Kα photons s-1 (4π)-1 is reported. Furthermore, experimental platforms for ultrafast X-ray diffraction and X-ray absorption and emission spectroscopy based on the reported source are described.

• Klíčová slova
• Cu Kα lines, ELI Beamlines, laser-driven sources, plasma X-ray sources, sub-picosecond sources, time-resolved experiments, ultrafast,
• Publikační typ
• časopisecké články MeSH

Hemoglobin is an oxygen-transport protein in red blood cells that interacts with multiple ligands, e.g., oxygen, carbon dioxide, carbon monoxide, and nitric oxide. Genetic variations in hemoglobin chains, such as those underlying sickle cell disease and thalassemias, present substantial clinical challenges. Here, we review the progress in research, including the use of allosteric modulators, pharmacological chaperones, and antioxidant treatments, which has begun to improve hemoglobin stability and oxygen affinity. According to UniProt (as of 7 August 2024), 819 variants of the α-hemoglobin subunit and 771 variants of the β-hemoglobin subunit have been documented, with over 116 classified as unstable. These data demonstrate the urgent need to develop variant-specific stabilizing options. Beyond small-molecule drugs/binders, novel protein-based strategies-such as engineered hemoglobin-binding proteins (including falcilysin, llama-derived nanobodies, and α-hemoglobin-stabilizing proteins)-offer promising new options. As our understanding of hemoglobin's structural and functional diversity grows, so does the potential for genotype-driven approaches. Continued research into hemoglobin stabilization and ligand-binding modification may yield more precise, effective treatments and pave the way toward effective strategies for hemoglobinopathies.

• Klíčová slova
• 2,3-bisphosphoglycerate (2,3-BPG), Bohr effect, allosteric regulation, genetic variants, hemoglobin (Hb), oxygen affinity, oxygen-binding properties, protein engineering, sickle cell disease (SCD), thalassemia,
• MeSH
• hemoglobinopatie farmakoterapie   genetika   metabolismus   MeSH
• hemoglobiny * genetika   chemie   metabolismus   MeSH
• lidé MeSH
• stabilita proteinů účinky léků   MeSH
• zvířata MeSH
• Check Tag
• lidé MeSH
• zvířata MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH
• Názvy látek
• hemoglobiny * MeSH