Novel ADAMTS13 mutations in a patient with congenital thrombotic thrombocytopenic purpura
Hematology, 2023 Congenital thrombotic thrombocytopenic purpura (TTP) is a rare autosomal recessive genetic disorder caused by mutations in the ADAMTS13 gene. Approximately 200 mutations of the ADAMTS-13 gene have been identified, although only a few have been ...
Zhitao Wang +6 more
doaj +1 more source
Effects of Fluorophore Attachment on Protein Conformation and Dynamics Studied by spFRET and NMR Spectroscopy. [PDF]
Chemistry, 2017 Sánchez-Rico C +4 more
europepmc +2 more sources
Limited proteolysis in the investigation of beta2-microglobulin amyloidogenic and fibrillar states. [PDF]
, 2005 Amyloid fibrils of patients treated with regular haemodialysis essentially consists of β2-microglobulin (β2-m) and its truncated species ΔN6β2-m lacking six residues at the amino terminus.
AMORESANO, ANGELA +4 more
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Aβ Secretion and Plaque Formation Depend on Autophagy
Cell Reports, 2013 Alzheimer’s disease (AD) is a neurodegenerative disease biochemically characterized by aberrant protein aggregation, including amyloid beta (Aβ) peptide accumulation. Protein aggregates in the cell are cleared by autophagy, a mechanism impaired in AD. To
Per Nilsson +9 more
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Cooperative Gating and Spatial Organization of Membrane Proteins through Elastic Interactions [PDF]
, 2007 Biological membranes are elastic media in which the presence of a transmembrane protein leads to local bilayer deformation. The energetics of deformation allow two membrane proteins in close proximity to influence each other's equilibrium conformation ...
Andrej Sali +4 more
core +5 more sources
Predicting Binding Free Energies of PDE2 Inhibitors. The Difficulties of Protein Conformation. [PDF]
Sci Rep, 2018 A congeneric series of 21 phosphodiesterase 2 (PDE2) inhibitors are reported. Crystal structures show how the molecules can occupy a ‘top-pocket’ of the active site.
Pérez-Benito L +3 more
europepmc +2 more sources
CHIMIA, 2011 The biological properties of a protein critically depend on its conformation, which can vary as a result of changes in conditions such as pH or following the addition of various substances.
Denis Prim +2 more
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Molecular dynamics as an approach to study prion protein misfolding and the effect of pathogenic mutations [PDF]
, 2011 Computer simulation of protein dynamics offers unique high-resolution information that complements experiment. Using experimentally derived structures of the natively folded prion protein (PrP), physically realistic dynamics and conformational changes ...
Daggett, Valerie, van der Kamp, Marc W
core +2 more sources
Structural Changes of RNA in Complex with Proteins in the SRP
Frontiers in Molecular Biosciences, 2018 The structural flexibility of RNA allows it to exist in several shapes and sizes. Thus, RNA is functionally diverse and is known to be involved in processes such as catalysis, ligand binding, and most importantly, protein recognition.
Janine K. Flores, Sandro F. Ataide
doaj +1 more source
Filter Retardation Assay for Detecting and Quantifying Polyglutamine Aggregates Using Caenorhabditis elegans Lysates [PDF]
, 2018 Protein aggregation is a hallmark of several neurodegenerative diseases and is associated with impaired protein homeostasis. This imbalance is caused by the loss of the protein's native conformation, which ultimately results in its aggregation or ...
Mata Cabana, Alejandro +3 more
core +2 more sources