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http://dx.doi.org/10.1002/anie.200501886 | DOI Listing |
Orphanet J Rare Dis
June 2024
Paediatric Rheumatology Unit, Hospital Sant Joan de Déu, Barcelona, Spain.
Background: Osteogenesis imperfecta (OI) is a rare disease characterized by low bone mass and bone fragility, associated with an increased risk of fractures, and skeletal and extra-skeletal symptoms that results in an impairment of health-related quality of life of OI patients. Since published studies on OI in Spain are limited, this study aimed to determine the epidemiology, assessed the disease burden, management and unmet needs of OI patients in Spain. Thirty-four experts in the management of patients with osteogenesis imperfecta completed two rounds of online consultation and reported real-life experience and data from Spanish hospitals.
View Article and Find Full Text PDFPhys Chem Chem Phys
June 2024
Department of Chemistry & Biochemistry, Thapar Institute of Engineering & Technology, Patiala-147004, Punjab, India.
Amyloid-β (Aβ) peptides aggregate spontaneously into various aggregating species comprising oligomers, protofibrils, and mature fibrils in Alzheimer's disease (AD). Disrupting β-sheet rich neurotoxic smaller soluble Aβ oligomers formed at early stages is considered a potent strategy to interfere with AD pathology. Previous experiments have demonstrated the inhibition of the early stages of Aβ aggregation by baicalein; however, the molecular mechanism behind inhibition remains largely unknown.
View Article and Find Full Text PDFNat Commun
October 2023
Department of Anesthesiology, Weill Cornell Medical College, New York, NY, USA.
Bcs1, a homo-heptameric transmembrane AAA-ATPase, facilitates folded Rieske iron-sulfur protein translocation across the inner mitochondrial membrane. Structures in different nucleotide states (ATPγS, ADP, apo) provided conformational snapshots, but the kinetics and structural transitions of the ATPase cycle remain elusive. Here, using high-speed atomic force microscopy (HS-AFM) and line scanning (HS-AFM-LS), we characterized single-molecule Bcs1 ATPase cycling.
View Article and Find Full Text PDFSci Rep
July 2023
Department of Physics and Astronomy, University of Missouri, Columbia, MO, 65211, USA.
Membrane proteins play critical roles in disease and in the disposition of many pharmaceuticals. A prime example is P-glycoprotein (Pgp) which moves a diverse range of drugs across membranes and out of the cell before a therapeutic payload can be delivered. Conventional structural biology methods have provided a valuable framework for comprehending the complex conformational changes underlying Pgp function, which also includes ATPase activity, but the lack of real-time information hinders understanding.
View Article and Find Full Text PDFMicroorganisms
August 2022
Colgate-Palmolive Technology Center, Piscataway, NJ 08854, USA.
An in situ study was conducted to examine the mode of action of a 0.454% stannous fluoride (SnF)-containing dentifrice in controlling the composition and properties of oral biofilm. Thirteen generally healthy individuals participated in the study.
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