Results 61 to 70 of about 4,786,755 (237)

Systemic Amyloidosis – Insights by Cardiovascular Magnetic Resonance [PDF]

, 2015
Systemic amyloidosis is the exemplar infiltrative, extracellular disease. Although it is a multi-organ disorder, cardiac involvement drives prognosis. Survival is worst in the AL amyloidosis subtype. It can affect any age and any race. There is no direct
Banypersad, SM
core  

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

The impact of limited healthcare access among patients with light chain and transthyretin amyloidosis: real-world survey during COVID-19 lockdown period in France

Orphanet Journal of Rare Diseases
Background The containment strategies during the COVID-19 pandemic between December 2019 and 2022 significantly disrupted the healthcare system. Cardiac amyloidosis has a poor prognosis and requires frequent follow-up in reference centres.
D. Guijarro, A. Jobbe-Duval, S. Aguilhon, F. Bauer, E. Donal, J. C. Eicher, J. Jeanneteau, B. Gellen, D. Kenizou, O. Lairez, B. Lequeux, D. Legallois, P. Réant, M. Salvat, M. F. Seronde, M. Kharoubi, A. Whereat, A. Farrugia, C. Taieb, A. Zaroui, T. Damy   +20 more
doaj   +1 more source

The diagnosis and characteristics of renal heavy-chain and heavy/light-chain amyloidosis and their comparison with renal light-chain amyloidosis [PDF]

Kidney International, 2013
Little is known about the rare entities of heavy- and light-chain amyloidosis (AHL) and heavy-chain amyloidosis (AH). Here, we report the renal and hematological characteristics, pathology, and outcome of 16 patients with renal AH/AHL (5 with AH and 11 with AHL) and compare them with 202 patients with renal light-chain amyloidosis (AL) diagnosed during
Nasr, Samih H., Said, Samar M., Valeri, Anthony M., Sethi, Sanjeev, Fidler, Mary E., Cornell, Lynn D., Gertz, Morie A., Dispenzieri, Angela, Buadi, Francis K., Vrana, Julie A., Theis, Jason D., Dogan, Ahmet, Leung, Nelson   +12 more
openaire   +2 more sources

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

Systemic light-chain amyloidosis revealed by progressive nail involvement, diffuse alopecia and sicca syndrome: report of an unusual case with a review of the literature. [PDF]

, 2014
Immunoglobulin light-chain (AL) amyloidosis is a form of systemic amyloidosis in which the fibrils are derived from monoclonal light chains. We report a case of a 66-year-old woman presenting with nail changes, parchment-like hand changes, progressive ...
Haneke, Eckart, Röcken, C, Borradori, Luca, Renker, Thekla   +3 more
core   +2 more sources

DNA Nanotechnology Meets Peptide and Protein Self‐Assembly

Chemistry – A European Journal, EarlyView.
Combining DNA nanotechnology with peptide and protein assembly provides complementary platforms for the rational engineering of functional biomaterials. This Perspective discusses the emerging field of self‐assembling DNA‐peptide and DNA‐protein hybrid systems that combine the structural precision and programmability of DNA nanotechnology with the ...
Marcel Hanke, Nanjundan Raghul, Adrian Keller, Veikko Linko, Verónica I. Dodero   +4 more
wiley   +1 more source

Predicting survival in light chain amyloidosis

Haematologica, 2019
In this issue of the Journal, Dittrich and coworkers report their systematic analysis of the performance of the three currently used staging systems for AL amyloidosis in a large population of 1,224 patients.[1][1] Their aim was to establish whether one of the available models had ...
Palladini G., Milani P., Merlini G.
openaire   +4 more sources

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  

Progression and prognostic significance of electrocardiographic findings in patients with cardiac amyloidosis

ESC Heart Failure, Volume 12, Issue 2, Page 809-818, April 2025.
Abstract Aims This study aimed to evaluate the change of the main electrocardiographic (ECG) characteristics and their prognostic role across the main subtypes of cardiac amyloidosis [light‐chain amyloidosis (AL) and hereditary (ATTRv) and wild‐type transthyretin amyloidosis (ATTRwt)].
Alessia Argirò, Mattia Zampieri, Carlotta Mazzoni, Carlo Fumagalli, Michela Baccini, Alessandra Mattei, Alberto Cipriani, Laura De Michieli, Aldostefano Porcari, Gianfranco Sinagra, Marco Merlo, Giacomo Tini, Beatrice Musumeci, Domitilla Russo, Pier Filippo Vianello, Marco Canepa, Roberto Licordari, Gianluca di Bella, Claudio Rapezzi, Federico Perfetto, Francesco Cappelli   +20 more
wiley   +1 more source