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Correlation of 99mTc-DPD bone scintigraphy with histological amyloid load in patients with ATTR cardiac amyloidosis

Amyloid: Journal of Protein Folding Disorders, 2023
Background The significance of measuring 99mTc-labelled-3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) in transthyretin (ATTR) cardiac amyloidosis has not been adequately studied. This single-centre observational study evaluated the correlation
M. Ungericht   +12 more
semanticscholar   +1 more source

Impact of tafamidis on myocardial strain in transthyretin amyloid cardiomyopathy

Amyloid: Journal of Protein Folding Disorders, 2022
Aims The impact of tafamidis on myocardial strain in patients with transthyretin amyloid cardiomyopathy (ATTR-CM) have been barely investigated. We aimed to determine tafamidis-induced changes using serial speckle tracking echocardiography and to ...
R. Rettl   +12 more
semanticscholar   +1 more source

Collagen inhibits phagocytosis of amyloid in vitro and in vivo and may act as a ‘don’t eat me’ signal

Amyloid: Journal of Protein Folding Disorders, 2022
Background Systemic amyloidosis refers to a group of protein misfolding disorders characterized by the extracellular deposition of amyloid fibrils in organs and tissues.
Joseph W. Jackson   +10 more
semanticscholar   +1 more source

Mass spectrometry-based proteomic analysis of parathyroid adenomas reveals PTH as a new human hormone-derived amyloid fibril protein

Amyloid: Journal of Protein Folding Disorders, 2021
Background Congo red-positive material was described in normal and diseased parathyroids (adenoma and hyperplasia) 50 years ago. However, the incidence and the clinical significance of such observation are unknown, and the causal fibril protein has never
M. Colombat   +11 more
semanticscholar   +1 more source

Somatostatin-derived amyloid deposition associated with duodenal neuroendocrine tumour (NET): a report of novel localised amyloidosis associated with NET

Amyloid: Journal of Protein Folding Disorders, 2021
Neuroendocrine tumours (NET) sometimes exhibit amyloid deposition [1]. Previously, we reported glucagon-derived localised amyloidosis associated with pancreatic NET [2].
S. Ichimata   +9 more
semanticscholar   +1 more source

Post-translational modification of amyloid a protein in patients with AA amyloidosis

Amyloid: Journal of Protein Folding Disorders, 2021
AA amyloidosis is a disease caused by extracellular deposition of insoluble β-pleated sheet fibrils composed of amyloid A (AA) protein, an amino (N)-terminal fragment of serum amyloid A (SAA).
B. Kluve-beckerman   +3 more
semanticscholar   +1 more source

Amyloid nomenclature 2024: update, novel proteins, and recommendations by the International Society of Amyloidosis (ISA) Nomenclature Committee

Amyloid: Journal of Protein Folding Disorders
The ISA Nomenclature Committee met at the XIX International Symposium of Amyloidosis in Rochester, MN, 27 May 2024. The in-person event was followed by many electronic discussions, resulting in the current updated recommendations.
J. N. Buxbaum   +7 more
semanticscholar   +1 more source

PRX004 in variant amyloid transthyretin (ATTRv) amyloidosis: results of a phase 1, open-label, dose-escalation study

Amyloid: Journal of Protein Folding Disorders
Background The investigational monoclonal antibody PRX004 is designed to specifically target and deplete TTR amyloid. Here, we report on the safety, tolerability, pharmacokinetics, pharmacodynamics and preliminary clinical activity of PRX004 in patients ...
O. Suhr   +8 more
semanticscholar   +1 more source

Genomic profiling in amyloid light-chain amyloidosis reveals mutation profiles associated with overall survival

Amyloid: Journal of Protein Folding Disorders, 2020
Background: Amyloid light chain (AL) amyloidosis is characterized by tissue deposition of amyloid fibres derived from immunoglobulin that can lead to irreversible organ damage. Information about genomic profiles of AL amyloidosis is lacking.
Xu-fei Huang   +12 more
semanticscholar   +1 more source

Cerebral amyloid angiopathy in a 51-year-old patient with embolization by dura mater extract and surgery for nasopharyngeal angiofibroma at age 17

Amyloid: Journal of Protein Folding Disorders, 2020
Cerebral amyloid angiopathy (CAA) is a small vessel disease, causing spontaneous intracerebral hemorrhage (ICH) in the elderly.
P. Caroppo   +10 more
semanticscholar   +1 more source

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