Results 41 to 50 of about 159,323 (346)

AL Amyloidosis [PDF]

Orphanet Journal of Rare Diseases, 2012
Abstract Definition of the disease AL amyloidosis results from extra-cellular deposition of fibril-forming monoclonal immunoglobulin (Ig) light chains (LC) (most commonly of lambda isotype) usually secreted by a small plasma cell clone.
Desport, Estelle, Bridoux, Franck, Sirac, Christophe, Delbes, Sébastien, Bender, Sébastien, Fernandez, Béatrice, Quellard, Nathalie, Lacombe, Corinne, Goujon, Jean-Michel, Lavergne, David, Abraham, Julie, Touchard, Guy, Fermand, Jean-Paul, Jaccard, Arnaud   +13 more
openaire   +4 more sources

Dissecting the Molecular Features of Systemic Light Chain (AL) Amyloidosis: Contributions from Proteomics

Medicina, 2021
Amyloidoses are characterized by aggregation of proteins into highly ordered amyloid fibrils, which deposit in the extracellular space of tissues, leading to organ dysfunction.
Paola Rognoni, Giulia Mazzini, Serena Caminito, Giovanni Palladini, Francesca Lavatelli   +4 more
doaj   +1 more source

QBP1 Peptide as a Potential Anti‐Amyloidogenic Therapy for Type 2 Diabetes: An In Vitro Study

Advanced Science, EarlyView.
The anti‐amyloidogenic peptide QBP1 effectively halts human islet amyloid polypeptide (hIAPP) aggregation, preventing the formation of toxic β‐structured intermediates. Through a combination of biophysical assays, molecular dynamics, and cell‐based studies, QBP1 is shown to preserve β‐cell viability and metabolic homeostasis, positioning it as a ...
María M. Tejero‐Ojeda, Ada Bernaus Vives, Michał Wojciechowski, Dinh Quoc Huy Pham, Mateusz Chwastyk, Mario Vallejo, Anna Novials, Douglas V. Laurents, Mariano Carrión‐Vázquez   +8 more
wiley   +1 more source

Multiorgan Dysfunction and Associated Prognosis in Transthyretin Cardiac Amyloidosis

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Background Transthyretin cardiac amyloidosis (ATTR‐CA) is a progressive and ultimately fatal cardiomyopathy. Biomarkers reflecting multiorgan dysfunction are of increasing importance in patients with heart failure; however, their significance in ATTR‐CA ...
Adam Ioannou, Christian Nitsche, Aldostefano Porcari, Rishi K. Patel, Yousuf Razvi, Muhammad U. Rauf, Ana Martinez‐Naharro, Lucia Venneri, Antonella Accietto, Lucrezia Netti, Francesco Bandera, Ruta Virsinskaite, Tushar Kotecha, Dan Knight, Aviva Petrie, Carol Whelan, Ashutosh Wechalekar, Helen Lachmann, Philip N. Hawkins, Julian D. Gillmore, Marianna Fontana   +20 more
doaj   +1 more source

The triphenylmethane dye brilliant blue G is only moderately effective at inhibiting amyloid formation by human amylin or at disaggregating amylin amyloid fibrils, but interferes with amyloid assays; Implications for inhibitor design. [PDF]

, 2019
The development of inhibitors of islet amyloid formation is important as pancreatic amyloid deposition contributes to type-2 diabetes and islet transplant failure.
Akter, Rehana, Bhatia, Surita R, Raleigh, Daniel P, Zheng, Bingqian, Zhyvoloup, Alexander   +4 more
core   +3 more sources

Carboranyl‐Curcuminoids for the Neutron Capture‐Based Treatment of Amyloid Aggregates in Alzheimer's Disease

Advanced Science, EarlyView.
The 10B‐enriched monocarbonyl analog of curcumin (BMAC) 10B‐9 enables site‐specific Boron Neutron Capture Therapy (BNCT) on amyloid‐β (Aβ) fibrils. Neutron irradiation induces histidine oxidation and fibril destabilization, as revealed by 1H‐NMR and FESEM analyses.
Sebastiano Micocci, Stefano Parisotto, Diego Alberti, Alberto Lanfranco, Valeria Bitonto, Polyssena Renzi, Martina Salmi, Andrea Magnani, Federica Dal Bello, Katiuscia Pagano, Laura Ragona, Nicoletta Protti, Annamaria Deagostino, Simonetta Geninatti Crich   +13 more
wiley   +1 more source

Cardiac transplantation in transthyretin amyloid cardiomyopathy: Outcomes from three decades of tertiary center experience

Frontiers in Cardiovascular Medicine, 2023
AimsTransthyretin cardiac amyloidosis (ATTR-CM) is a progressive and fatal cardiomyopathy. Treatment options in patients with advanced ATTR-CM are limited to cardiac transplantation (CT).
Yousuf Razvi, Aldostefano Porcari, Aldostefano Porcari, Concetta Di Nora, Rishi K. Patel, Adam Ioannou, Muhammad U. Rauf, Ambra Masi, Steven Law, Liza Chacko, Tamer Rezk, Sriram Ravichandran, Janet Gilbertson, Dorota Rowczenio, Iona J. Blakeney, Nandita Kaza, David F. Hutt, Helen Lachmann, Ashutosh Wechalekar, William Moody, Sern Lim, Colin Chue, Carol Whelan, Lucia Venneri, Ana Martinez-Naharro, Marco Merlo, Gianfranco Sinagra, Ugolino Livi, Philip Hawkins, Marianna Fontana, Julian D. Gillmore   +30 more
doaj   +1 more source

Sex-specific effects of microbiome perturbations on cerebral Aβ amyloidosis and microglia phenotypes. [PDF]

, 2019
We demonstrated that an antibiotic cocktail (ABX)-perturbed gut microbiome is associated with reduced amyloid-β (Aβ) plaque pathology and astrogliosis in the male amyloid precursor protein (APP)SWE /presenilin 1 (PS1)ΔE9 transgenic model of Aβ ...
Baufeld, Caroline, Butovsky, Oleg, Dodiya, Hemraj B, Gilbert, Jack A, Gottel, Neil, Kuntz, Thomas, Leibowitz, Jeffrey, Shaik, Shabana M, Sisodia, Sangram S, Zhang, Xiaoqiong, Zhang, Xulun   +10 more
core   +1 more source

Brain and Liver Dual‐Targeting Oridonin Nanoparticles to Enhance Aβ Clearance for Alzheimer's Disease Therapy

Advanced Science, EarlyView.
We developed a nanoparticle named OAF, which simultaneously targeted to both the brain and liver via the transferrin receptor 1 (TfR1) receptor, promoting lipoprotein receptor‐related protein 1 (LRP1) expression to enhance amyloid‐beta (Aβ) clearance. In AD mice model, OAF significantly reduced Aβ deposition and cognitive impairment, while a mitigating
Wenshuai Gong, Wenting Hui, Sai Qiao, Qifeng Ji, Miao Liu, Bangle Zhang, Daozhou Liu, Yumei Wu, Siyuan Zhou   +8 more
wiley   +1 more source

Real-world treatment management in hereditary transthyretin amyloidosis – an experience report and proposal for therapy switch decision criteria

Neurological Research and Practice
Background Hereditary transthyretin amyloidosis is a rapidly progressive and lethal disease. Thanks to the increasing number of disease-modifying treatments, prognosis has improved significantly.
Duc Chu Dieu, Helena F. Pernice, Harisa Muratovic, Paul J. Wetzel, Gina Barzen, Nicolas W. Wieder, Stefanie M. Werhahn, Bettina Heidecker, Sebastian Spethmann, Katrin Hahn   +9 more
doaj   +1 more source