Results 21 to 30 of about 6,309 (194)

Catheter Ablation and Device Therapy in Patients With Transthyretin Amyloid Cardiomyopathy: A Review of Non-Pharmacological Therapy. [PDF]

J Arrhythm
Transthyretin amyloid cardiomyopathy (ATTR‐CM) frequently coexists with atrial fibrillation, atrial flutter, and atrial tachycardia as well as occasionally ventricular arrhythmias and often conduction abnormalities, necessitating management for arrhythmias.
Kanazawa H, Hoshiyama T, Kaneko S, Kawahara Y, Tsuruta Y, Tsurusaki Y, Matsunaga K, Tamanoi S, Kuyama N, Usuku H, Yamamoto E, Izumiya Y, Tsujita K.   +12 more
europepmc   +2 more sources

Single-centre experience on transthyretin familial amyloid polyneuropathy : case series and literature review [PDF]

, 2018
Familial amyloid polyneuropathy (FAP) is a most often length-dependent axonal neuropathy, often part of a multisystem disorder also affecting other organs, such as cardiac, gastrointestinal, genitourinary, renal, meningeal and eye tissue.
De Bleecker, Jan, De Pauw, Michel, Martens, Broes   +2 more
core   +2 more sources

Peripheral Blood Cell Gene Expression Diagnostic for Identifying Symptomatic Transthyretin Amyloidosis Patients: Male and Female Specific Signatures [PDF]

, 2016
BACKGROUND: Early diagnosis of familial transthyretin (TTR) amyloid diseases remains challenging because of variable disease penetrance. Currently, patients must have an amyloid positive tissue biopsy to be eligible for disease-modifying therapies ...
Buxbaum, J., Coelho, T., Gelbart, T., Kelly, J., Kurian, S., Novais, M., Salomon, D., Whisenant, T.   +7 more
core   +1 more source

Extrapolation of Survival Benefits in Patients with Transthyretin Amyloid Cardiomyopathy Receiving Tafamidis: Analysis of the Tafamidis in Transthyretin Cardiomyopathy Clinical Trial

Cardiology and Therapy, 2020
Introduction In the Tafamidis in Transthyretin Cardiomyopathy Clinical Trial (ATTR-ACT; ClinicalTrials.gov number NCT01994889), tafamidis reduced the risk of all-cause mortality in patients with transthyretin amyloid cardiomyopathy (ATTR-CM) by 30 ...
Benjamin Li, Jose Alvir, Michelle Stewart   +2 more
doaj   +1 more source

Long-term effects of tafamidis for the treatment of transthyretin familial amyloid polyneuropathy [PDF]

, 2013
Tafamidis, a transthyretin (TTR) kinetic stabilizer, delayed neuropathic progression in patients with Val30Met TTR familial amyloid polyneuropathy (TTR-FAP) in an 18-month randomized controlled trial (study Fx-005).
Ana Martins da Silva, Donna R. Grogan, Hartmut H.-J. Schmidt, Isabel Conceiçao, Jason Chan, Jeff Packman, Jeffery W. Kelly, Luis F. Maia, Márcia W. Cruz, Ole B. Suhr, Pedro Trigo, Richard Labaudinière, Teresa Coelho, Violaine Planté-Bordeneuve   +13 more
core   +1 more source

A cationic tetrapyrrole inhibits toxic activities of the cellular prion protein [PDF]

, 2016
Prion diseases are rare neurodegenerative conditions associated with the conformational conversion of the cellular prion protein (PrPC) into PrPSc, a self-replicating isoform (prion) that accumulates in the central nervous system of affected individuals.
Biasini, Emiliano, Borsello, Tiziana, Castilla, Joaqu\uedn, Cerovic, Milica, Cimini, Sara, Elezgarai, Saioa R., Gobbi, Marco, Massignan, Tania, Moreno, Jorge, Negro, Alessandro, Nonno, Romolo, Restelli, Elena, Riccardi, Geraldina, Sangiovanni, Valeria, Stincardini, Claudia, Stravalaci, Matteo, Vanni, Ilaria   +16 more
core   +2 more sources

Biophysical characterization and modulation of Transthyretin Ala97Ser

Annals of Clinical and Translational Neurology, 2019
Objective Ala97Ser (A97S) is the major transthyretin (TTR) mutation in Taiwanese patients of familial amyloid polyneuropathy (FAP), characterized by a late‐onset but rapidly deteriorated neuropathy.
Yo‐Tsen Liu, Yueh‐Jung Yen, Frans Ricardo, Yu Chang, Pei‐Hao Wu, Shing‐Jong Huang, Kon‐Ping Lin, Tsyr‐Yan Yu   +7 more
doaj   +1 more source

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

, 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 ...
Bellotti, Vittorio, Corazza, Alessandra, Faravelli, Giulia, Gillmore, Julian D, Giorgetti, Sofia, Hawkins, Philip N, Mangione, P. Patrizia, Pepys, Mark B, Porcari, Riccardo, Raimondi, Sara, Taylor, Graham W, Verona, Guglielmo   +11 more
core   +2 more sources

Improvements in Efficacy Measures With Tafamidis in the Tafamidis in Transthyretin Cardiomyopathy Clinical Trial

JACC: Advances, 2022
Transthyretin amyloid cardiomyopathy (ATTR-CM) is a fatal disease. Tafamidis was approved to treat patients with ATTR-CM based on findings from the ATTR-ACT (Tafamidis in Transthyretin Cardiomyopathy Clinical Trial).This post hoc analysis examined the proportion of patients who experienced improved efficacy measures through 30 months of treatment with ...
Hanna, Mazen, Fine, Nowell M., Gundapaneni, Balarama, Sultan, Marla B., Witteles, Ronald M.   +4 more
openaire   +2 more sources

Familial amyloidosis with polyneuropathy type 1 caused by transthyretin mutation Val50Met (Val30Met): 4 cases in a non-endemic area [PDF]

, 2018
Introduction: Transthyretin-related familial amyloid polyneuropathy (TTR-FAP) typically arises as an autonomic neuropathy primarily affecting small fibres and it occurs in adult patients in their second or third decades of life. It progresses rapidly and
Andrés, N., Martí Massó, José Félix, Poza Aldea, Juan José   +2 more
core   +3 more sources