Results 41 to 50 of about 99,289 (260)

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

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

Early Retinal UCHL1 Dysregulation Coupled With Synaptic Loss Reflects Alzheimer's Disease Severity

Advanced Science, EarlyView.
This study identifies synapse‐enriched deubiquitinase UCHL1 as an early Aβ‐responsive regulator of retinal synaptopathy in Alzheimer's disease. Retinal UCHL1 loss accompanies excitatory synapse degeneration, p75NTR activation, and neuroinflammation, and predicts Braak stage and cognitive decline. Aβ42 fibrils trigger synaptic and UCHL1 depletion before
Altan Rentsendorj, Jean‐Philippe Vit, Alexandre Hutton, Yosef Koronyo, Saba Shahin, Edward Robinson, Ernesto Barron, Anthony Rodriguez, Ousman Jallow, Julia Sheyn, Bhakta Prasad Gaire, Lalita Subedi, Miyah R. Davis, Barathan Gnanabharathi, Alexander V. Ljubimov, Stuart L. Graham, Vivek K. Gupta, Mehdi Mirzaei, Debra Hawes, Katlin Silm, Keith L. Black, Lon S. Schneider, Alfredo A. Sadun, Jesse G. Meyer, Dieu‐Trang Fuchs, Maya Koronyo‐Hamaoui   +25 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

Natural history and outcome in systemic AA amyloidosis [PDF]

, 2007
BACKGROUND:Deposition of amyloid fibrils derived from circulating acute-phase reactant serum amyloid A protein (SAA) causes systemic AA amyloidosis, a serious complication of many chronic inflammatory disorders.
Goodman, HJB, Lachmann, HJ, Gillmore, JD, Hawkins, PN, Gilbertson, JA, Gallimore, JR, Sabin, CA   +6 more
core  

Localised Mass-forming Amyloidosis of the Genitourinary Tract Mimics Urothelial Carcinoma: Two Cases of Mistaken Identity

, 2021
Amyloidosis of the lower genitourinary tract is an extremely rare condition and often mimics urothelial malignancy. In this paper, we report two cases of ureteral and urethral amyloidosis that initially presented as urothelial carcinoma and were ...
Aiyapa Aruna Ajjikuttira, Eric Chung, Matthew John Roberts, Darius Ashrafi, Pranav Sharma, Handoo Rhee   +5 more
core   +1 more source

Aberrant GRP78 Phase Transition Sustains Endothelial IRE1α Signaling and Drives Blood–Brain Barrier Failure in Cerebral Amyloid Angiopathy

Advanced Science, EarlyView.
Vascular Aβ40 corrupts GRP78 phase behavior in brain endothelial cells, sustaining IRE1α–TRAF2–JNK signaling and driving apoptosis, tight junction loss, and blood–brain barrier failure in cerebral amyloid angiopathy. Pharmacological IRE1α inhibition restores vascular integrity, reduces leakage, and improves functional outcomes, revealing a targetable ...
Honglin Zheng, Qiang Li, Haiyang Luo, Yapei Yuan, Na Zhang, Yongting Lu, Suying Duan, Jieshi Zhong, Hang Zhang, Chenyang Liu, Yaochong Zhang, Wenzhuo Zhao, Yaxuan Song, Tuo Wang, Si Yan, Yapeng Li, Han Liu, Yuan Gao, Zongping Xia, Yuming Xu   +19 more
wiley   +1 more source

Eprodisate for the treatment of renal disease in AA amyloidosis [PDF]

, 2007
Background: Amyloid A (AA) amyloidosis is a complication of chronic inflammatory conditions that develops when proteolytic fragments of serum amyloid A protein (SAA) are deposited in tissues as amyloid fibrils.
Dember, L.M., Hazenberg, B.P.C., Garceau, D., Puéchal, X., Livneh, A., Lesnyak, O., Skinner, Martha, Lesnyak, Olga, Livneh, Avi, Butrimiene, I., Merlini, G., Hauck, Wendy, Obici, L., Dember, Laura M., Gorevic, Peter D., Skinner, M., Butrimiene, Irena, Hawkins, Philip N., Balshaw, Robert, Hazenberg, Bouke P. C.; id_orcid, Lachmann, Helen J., Obici, Laura, Gorevic, P.D., Puechal, Xavier, Hazenberg, Bouke P. C., Hawkins, P.N., Garceau, Denis, Balshaw, R., Lachmann, H.J., Merlini, Giampaolo, Hauck, W.   +30 more
core  

Mechanisms underlying the initiation of cerebral betaamyloidosis and neurofibrillary tau pathology : new insights form transgenic mice [PDF]

, 2006
Numerous neurodegenerative disorders result from the aggregation of proteins that misfold and accumulate as fibrillar amyloid deposits in selectively vulnerable regions of the central nervous system.
Bolmont, Tristan
core   +1 more source

Radical Resection of the Third Portion of the Duodenum for Secondary Aortoduodenal Fistula

Annals of Gastroenterological Surgery, EarlyView.
Secondary aortoduodenal fistula most commonly involves the third portion of the duodenum and requires definitive management of both vascular and gastrointestinal components. We demonstrate a step‐by‐step technique for radical duodenal resection and reconstruction performed in structured collaboration with cardiovascular surgeons.
Koji Kubota, Akira Shimizu, Tsuyoshi Notake, Tatsuichiro Seto, Yuji Soejima   +4 more
wiley   +1 more source