Results 71 to 80 of about 90,332 (308)

Inhibition of the mechano-enzymatic amyloidogenesis of transthyretin: role of ligand affinity, binding cooperativity and occupancy of the inner channel

Scientific Reports, 2017
Dissociation of the native transthyretin (TTR) tetramer is widely accepted as the critical step in TTR amyloid fibrillogenesis. It is modelled by exposure of the protein to non-physiological low pH in vitro and is inhibited by small molecule compounds ...
Guglielmo Verona, P. Patrizia Mangione, Sara Raimondi, Sofia Giorgetti, Giulia Faravelli, Riccardo Porcari, Alessandra Corazza, Julian D. Gillmore, Philip N. Hawkins, Mark B. Pepys, Graham W. Taylor, Vittorio Bellotti   +11 more
doaj   +1 more source

Atraumatic splenic rupture in amyloidosis

, 2009
BACKGROUND: Splenic involvement in amyloidosis is rather frequent (5-10%). An atraumatic rupture of the affected spleen is however an extremely rare event.
Candinas, Daniel, Schoepfer, Alain, Renzulli, Pietro, Mueller, Esther   +3 more
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Primary Bladder Amyloidosis Mimicking Bladder Cancer: A Rare Case Report [PDF]

Bladder amyloidosis is a rare condition that can mimic malignancy clinically and radiologically. A 44-year-old female patient was admitted to our clinic with macroscopic hematuria and dysuria.
BOZKURT, OZAN, Volkan Şen, SİNİRSİZ, CAN, Can Sinirsiz, TUNA, EMİNE BURÇİN, ŞEN, VOLKAN, Emine Burçin Tuna, Ozan Bozkurt   +7 more
core   +1 more source

A Multifaceted Interplay Among Hemophagocytosis, Interleukin‐18, and Type I Interferon Distinguishes Still Disease From Other Autoinflammatory Diseases

Arthritis &Rheumatology, EarlyView.
Objective The unknown pathophysiology and the lack of specific features for systemic juvenile idiopathic arthritis and adult‐onset Still disease (collectively known as Still disease; SD) delay diagnosis and appropriate treatment. The goal of this study was to identify features and mechanisms that distinguish SD from other systemic autoinflammatory ...
Yvonne M. Mueller, Charlotte Girard, Harmen J. G. van de Werken, Stefan J. Erkeland, Dwin G. B. Grashof, Laurie Vaillant, Renske J. H. den Dekker, Iris van Zuijen, Sharon Veenbergen, Fanny Coffin, Eric Bindels, Yvan Jamilloux, Katerina Laskari, Vassili Soumelis, Bruno Fautrel, Cem Gabay, Peter D. Katsikis   +16 more
wiley   +1 more source

Revised renal stratification and progression models for predicting long-term renal outcomes in immunoglobulin light chain amyloidosis

Haematologica
Renal prognosis in light-chain amyloidosis (AL) is determined by categorizing patients into three renal stages at diagnosis and assessing Renal Response or Renal Progression following chemotherapy after 6 months.
Muhammad Umaid Rauf, Steven Law, Marisa Santostefano, Philip N. Hawkins, Aviva Petrie, Francesco Cappelli, Federico Perfetto, Yousuf Razvi, Aldostefano Porcari, Sriram Ravichandran, Adam Ioannou, Joshua Bomsztyk, Alessia Argirò, Costanza Gaudio, Elisabetta Antonioli, Alessandro Barilaro, Marco Delsante, Vittorio Di Maso, Maria G. Chiappini, Olabisi Ogunbiyi, Oliver C. Cohen, Ana Martinez-Naharro, Carol Whelan, Helen J. Lachmann, Ashutosh D. Wechalekar, Federico Alberici, Marianna Fontana, Marco Allinovi, Julian D. Gillmore   +28 more
doaj   +1 more source

Systemic amyloidosis in England: an epidemiological study.

, 2013
Epidemiological studies of systemic amyloidosis are scarce and the burden of disease in England has not previously been estimated. In 1999, the National Health Service commissioned the National Amyloidosis Centre (NAC) to provide a national clinical ...
Smith, CJ, Banypersad, SM, Whelan, CJ, Venner, CP, Dungu, J, Pinney, JH, Lachmann, HJ, Taube, JB, Gillmore, JD, Gibbs, SD, Hawkins, PN, Wechalekar, AD   +11 more
core  

Decoding RNA regulation: Challenges and opportunities for RNA‐based therapies in Europe

British Journal of Clinical Pharmacology, EarlyView.
Abstract RNA‐based medicinal products represent a promising frontier in personalised medicine, offering sequence‐specific disease targeting at various molecular levels, yet their clinical translation in the European Union (EU) may be hindered by regulatory uncertainty around definitions and evidence requirements; this study therefore aims to identify ...
Olivia C. Lewis, Christine Leopold, Joyce M. Hoek, Raymond M. Schiffelers, Marie L. De Bruin   +4 more
wiley   +1 more source

Reliability and validity of the Transthyretin Amyloidosis – Quality of Life (ATTR-QOL) Questionnaire impact scales

Journal of Patient-Reported Outcomes
Background The diversity of disease phenotypes associated with transthyretin (ATTR) amyloidosis poses challenges for measurement of health outcomes. The Transthyretin Amyloidosis – Quality of Life (ATTR-QOL) Questionnaire is a disease-specific patient ...
Andrew Lovley, Kristen Hsu, Kaitlin LaGasse, Isabelle Lousada, Kristen L. McCausland, Michelle K. Carty, Sabrina Rebello, Jakob B. Bjorner   +7 more
doaj   +1 more source

Tafamidis Reduces Death and Hospitalization for Acute Heart Failure in Octogenarian Patients With Transthyretin Cardiac Amyloidosis: A Propensity Score–Weighted Cohort Study

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Background Transthyretin cardiac amyloidosis results from the deposition of misfolded amyloid fibrils in cardiac tissue, leading to progressive heart failure.
Louise Zmuda, Amira Zaroui, Mounira Kharoubi, Emmanuelle Boutin, Frédéric Roca, Silvia Oghina, Emmanuel Teiger, Marie Laurent, Florence Canoui‐Poitrine, Thibaud Damy, Amaury Broussier   +10 more
doaj   +1 more source

IN SILICO STUDIES OF AMYLOID FORMATION AND AMYLOID STABILITY [PDF]

, 2020
The term “Amyloid” describes the precursor proteins misfolded and aggregated into fibril-like structures that are built by cross β-sheet subunit. The disease caused by depositing amyloid fibril in tissues and organs is called amyloidosis. Amyloidosis can
Wang, Wenhua
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