Search Results - "Benson, Merrill"
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Liver transplantation and transthyretin amyloidosis
Published in Muscle & nerve (01-02-2013)“…Liver transplantation as a specific treatment of transthyretin amyloidosis was first performed in 1990. The rationale for this treatment was that removal of…”
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Diagnosis, Prognosis, and Therapy of Transthyretin Amyloidosis
Published in Journal of the American College of Cardiology (01-12-2015)“…Abstract Transthyretin amyloidosis is a fatal disorder that is characterized primarily by progressive neuropathy and cardiomyopathy. It occurs in both a mutant…”
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Amyloid nomenclature 2020: update and recommendations by the International Society of Amyloidosis (ISA) nomenclature committee
Published in Amyloid (01-10-2020)“…The ISA Nomenclature Committee met electronically before and directly after the XVII ISA International Symposium on Amyloidosis, which, unfortunately, had to…”
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Amyloid nomenclature 2018: recommendations by the International Society of Amyloidosis (ISA) nomenclature committee
Published in Amyloid (02-10-2018)“…The nomenclature committee of the International Society of Amyloidosis (ISA) meets every second year to discuss and formulate recommendations. The conclusions…”
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Amyloid fibril proteins and amyloidosis: chemical identification and clinical classification International Society of Amyloidosis 2016 Nomenclature Guidelines
Published in Amyloid (01-10-2016)“…The Nomenclature Committee of the International Society of Amyloidosis (ISA) met during the XVth Symposium of the Society, 3 July-7 July 2016, Uppsala, Sweden,…”
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Nomenclature 2014: Amyloid fibril proteins and clinical classification of the amyloidosis
Published in Amyloid (01-12-2014)Get full text
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Inotersen therapy of transthyretin amyloid cardiomyopathy
Published in Amyloid (02-01-2020)“…Background: Cardiomyopathy is a major cause of death in patients with systemic transthyretin amyloidosis. Long term effect of therapy designed to inhibit…”
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The molecular biology and clinical features of amyloid neuropathy
Published in Muscle & nerve (01-10-2007)“…Neuropathy is often a major manifestation of systemic amyloidosis. It is most frequently seen in patients with hereditary transthyretin (TTR) amyloidosis, but…”
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Amyloid seeding of transthyretin by ex vivo cardiac fibrils and its inhibition
Published in Proceedings of the National Academy of Sciences - PNAS (17-07-2018)“…Each of the 30 human amyloid diseases is associated with the aggregation of a particular precursor protein into amyloid fibrils. In transthyretin amyloidosis…”
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Guidelines and new directions in the therapy and monitoring of ATTRv amyloidosis
Published in Amyloid (03-07-2022)“…The recent approval of three drugs for the treatment of amyloid transthyretin (ATTR) amyloidosis, both hereditary and wild-type, has opened a new era in the…”
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Inotersen treatment for ATTR amyloidosis
Published in Amyloid (29-03-2019)Get full text
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POTENTIAL REVERSAL OF TRANSTHYRETIN AMYLOID CARDIOMYOPATHY WITH TTR SPECIFIC ANTISENSE OLIGONUCLEOTIDE THERAPY
Published in Journal of the American College of Cardiology (10-03-2018)“…In the majority of patients with ATTR cardiomyopathy death is due to progressive cardiac failure from restrictive cardiomyopathy. Since TTR is synthesized…”
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Detection of Circulating Transthyretin Amyloid Aggregates in Plasma: A Novel Biomarker for Transthyretin Amyloidosis
Published in Circulation (New York, N.Y.) (21-05-2024)Get full text
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14
Progression of transthyretin amyloid neuropathy after liver transplantation
Published in Neurology (27-07-2010)“…To biochemically characterize peripheral nerve amyloid in subjects with transthyretin (TTR) amyloidosis and assess effect of orthotopic liver transplantation…”
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Safety and efficacy of a TTR specific antisense oligonucleotide in patients with transthyretin amyloid cardiomyopathy
Published in Amyloid (02-10-2017)“…Objectives: Cardiomyopathy is a major cause of death in both the hereditary form of transthyretin (TTR) amyloidosis and the sporadic late-age-onset…”
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Editorial Commentary: Advances in transthyretin amyloid cardiomyopathy
Published in Trends in cardiovascular medicine (01-01-2021)“…First described as an autosomal dominant inherited trait with adult onset peripheral neuropathy as the major clinical manifestation, it is now recognized as a…”
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LECT2 amyloidosis
Published in Kidney international (01-05-2010)“…LECT2 amyloidosis is the latest systemic type of amyloidosis to be described. It was discovered in patients with nephrotic syndrome and renal failure and is…”
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Transthyretin amyloidosis: a little history of hereditary amyloidosis
Published in Amyloid (16-03-2017)Get full text
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Hereditary systemic immunoglobulin light-chain amyloidosis
Published in Blood (21-05-2015)“…Several members of a family died from renal failure as a result of systemic amyloidosis. Extensive studies to detect previously documented gene mutations…”
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Suppressing transthyretin production in mice, monkeys and humans using 2nd-Generation antisense oligonucleotides
Published in Amyloid (01-09-2016)“…Transthyretin amyloidosis (ATTR amyloidosis) is a rare disease that results from the deposition of misfolded transthyretin (TTR) protein from the plasma into…”
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