Results 21 to 30 of about 159,323 (346)

Inhibition by small-molecule ligands of formation of amyloid fibrils of an immunoglobulin light chain variable domain. [PDF]

, 2015
Overproduction of immunoglobulin light chains leads to systemic amyloidosis, a lethal disease characterized by the formation of amyloid fibrils in patients' tissues. Excess light chains are in equilibrium between dimers and less stable monomers which can
Brumshtein, Boris, Cascio, Duilio, Eisenberg, David S, Esswein, Shannon R, Ly, Alan T, Phillips, Martin L, Salwinski, Lukasz, Sawaya, Michael R   +7 more
core   +1 more source

Clinical Amyloid Typing by Proteomics: Performance Evaluation and Data Sharing between Two Centres

Molecules, 2021
Amyloidosis is a relatively rare human disease caused by the deposition of abnormal protein fibres in the extracellular space of various tissues, impairing their normal function. Proteomic analysis of patients’ biopsies, developed by Dogan and colleagues
Diana Canetti, Francesca Brambilla, Nigel B. Rendell, Paola Nocerino, Janet A. Gilbertson, Dario Di Silvestre, Andrea Bergamaschi, Francesca Lavatelli, Giampaolo Merlini, Julian D. Gillmore, Vittorio Bellotti, Pierluigi Mauri, Graham W. Taylor   +12 more
doaj   +1 more source

Uncovering the Mechanism of Aggregation of Human Transthyretin. [PDF]

, 2015
The tetrameric thyroxine transport protein transthyretin (TTR) forms amyloid fibrils upon dissociation and monomer unfolding. The aggregation of transthyretin has been reported as the cause of the life-threatening transthyretin amyloidosis.
Cascio, Duilio, Eisenberg, David S, Jiang, Lin, Johnson, Lisa M, Liang, Wilson Y, Riek, Roland, Ruchala, Piotr, Saelices, Lorena, Sawaya, Michael R, Whitelegge, Julian   +9 more
core   +2 more sources

Marked progress in AL amyloidosis survival: a 40-year longitudinal natural history study

Blood Cancer Journal, 2021
The recent decades have ushered in considerable advancements in the diagnosis and treatment of systemic light chain (AL) amyloidosis. As disease outcomes improve, AL amyloidosis-unrelated factors may impact mortality. In this study, we evaluated survival
Andrew Staron, Luke Zheng, Gheorghe Doros, Lawreen H. Connors, Lisa M. Mendelson, Tracy Joshi, Vaishali Sanchorawala   +6 more
doaj   +1 more source

Human central nervous system (CNS) ApoE isoforms are increased by age, differentially altered by amyloidosis, and relative amounts reversed in the CNS compared with plasma [PDF]

, 2016
The risk of Alzheimer's disease (AD) is highly dependent on apolipoprotein-E (apoE) genotype. The reasons for apoE isoform-selective risk are uncertain; however, both the amounts and structure of human apoE isoforms have been hypothesized to lead to ...
Baker-Nigh, Alaina T, Bateman, Randall J, Bollinger, James G, Cairns, Nigel J, Franklin, Erin E, Holtzman, David, Jiang, Hong, Kasten, Tom, Liao, Fan, Mawuenyega, Kwasi G, Morris, John C, Ovod, Vitaliy   +11 more
core   +3 more sources

Proteomic Analysis for the Diagnosis of Fibrinogen Aα-chain Amyloidosis

Kidney International Reports, 2019
Introduction: Hereditary fibrinogen Aα-chain (AFib) amyloidosis is a relatively uncommon renal disease associated with a small number of pathogenic fibrinogen Aα (FibA) variants; wild-type FibA normally does not result in amyloid deposition.
Graham W. Taylor, Janet A. Gilbertson, Rabya Sayed, Angel Blanco, Nigel B. Rendell, Dorota Rowczenio, Tamer Rezk, P. Patrizia Mangione, Diana Canetti, Paul Bass, Philip N. Hawkins, Julian D. Gillmore   +11 more
doaj   +1 more source

Electromechanical dissociation of left atrium in patients with Cardiac Amyloidosis by Magnetic Resonance: Prognostic and clinical correlates

International Journal of Cardiology: Heart & Vasculature, 2020
Objectives: Left atrial (LA) function is an important marker of hemodynamic status in cardiac amyloidosis (CA), and its characterization may provide relevant prognostic information.
Giovanni Donato Aquaro, Sofia Morini, Chrysanthos Grigoratos, Giulia Taborchi, Gianluca Di Bella, Raffaele Martone, Elisa Vignini, Michele Emdin, Iacopo Olivotto, Federico Perfetto, Francesco Cappelli   +10 more
doaj   +1 more source

Comparative Outcomes of a Transthyretin Amyloid Cardiomyopathy Cohort Versus Patients With Heart Failure With Preserved Ejection Fraction Enrolled in the TOPCAT Trial

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease, 2023
Background Transthyretin cardiac amyloidosis (ATTR‐CM), found in 6% to 15% of cohorts with heart failure with preserved ejection fraction, has long been considered a rare disease with poor prognosis.
Morris M. Kim, Mark Prasad, Yunwoo Burton, Clinton M. Kolseth, Yuanzi Zhao, Pranav Chandrashekar, Babak Nazer, Ahmad Masri   +7 more
doaj   +1 more source

A nationwide assessment of hepatocellular adenoma resection: Indications and pathological discordance

Hepatology Communications, EarlyView., 2022
Abstract Hepatocellular adenomas (HCAs) are benign liver tumors associated with bleeding or malignant transformation. Data on the indication for surgery are scarce. We analyzed indications and outcome of patients operated for HCAs < 50 mm compared to HCAs ≥ 50 mm. Changes in final postoperative diagnosis were assessed.
Martijn P. D. Haring, Arthur K. E. Elfrink, Christiaan A. J. Oudmaijer, Paul C. M. Andel, Alicia Furumaya, Nenke de Jong, Colin J. J. M. Willems, Thijs Huits, Julie M. L. Sijmons, Eric J. T. Belt, Koop Bosscha, Esther C. J. Consten, Mariëlle M. E. Coolsen, Peter van Duijvendijk, Joris I. Erdmann, Paul Gobardhan, Robbert J. de Haas, Tjarda van Heek, Hwai‐Ding Lam, Wouter K. G. Leclercq, Mike S. L. Liem, Hendrik A. Marsman, Gijs A. Patijn, Türkan Terkivatan, Babs M. Zonderhuis, Izaak Quintus Molenaar, Wouter W. te Riele, Jeroen Hagendoorn, Alexander F. M. Schaapherder, Jan N. M. IJzermans, Carlijn I. Buis, Joost M. Klaase, Koert P. de Jong, Vincent E. de Meijer, the Dutch Benign Liver Tumor Group, R. B. Takkenberg, J. Verheij, O. M. van Delden, J. I. Erdmann, B. V. van Rosmalen, A. Furumaya, G. Kazemier, D. Ramsoekh, J. N. M. IJzermans, R. A. de Man, M. D. Doukas, M. G. Thomeer, A. J. Klompenhouwer, A. F. M. Schaapherder, M. E. Tushuizen, M. J. Coenraad, A. S. L. P. Crobach, M. C. Burgmans, S. Feshtali, M. M. E. Coolsen, M. Kramer, J. C. Beckervordersandforth, I. V. Samarska, C. van der Leij, R. Miclea, J. H. W. de Wilt, P. B. van den Boezem, E. T. T. L. Tjwa, I. Munsterman, S. Vos, S. van Koeverden, V. E. de Meijer, F. J. C. Cuperus, R. J. de Haas, E. W. Duiker, M. P. D. Haring   +70 more
wiley   +1 more source

Renal Amyloidosis Associated With 5 Novel Variants in the Fibrinogen A Alpha Chain Protein

Kidney International Reports, 2017
Fibrinogen A alpha chain amyloidosis is an autosomal dominant disease associated with mutations in the fibrinogen A alpha chain (FGA) gene, and it is the most common cause of hereditary renal amyloidosis in the UK.
Dorota Rowczenio, Maria Stensland, Gustavo A. de Souza, Erik H. Strøm, Janet A. Gilbertson, Graham Taylor, Nigel Rendell, Shane Minogue, Yvonne A. Efebera, Helen J. Lachmann, Ashutosh D. Wechalekar, Philip N. Hawkins, Ketil R. Heimdal, Kristian Selvig, Inger K. Lægreid, Nathalie Demoulin, Selda Aydin, Julian D. Gillmore, Tale N. Wien   +18 more
doaj   +1 more source