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Dialysis related Amyloidosis

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https://www.readbyqxmd.com/read/28536682/a-case-of-intramural-coronary-amyloidosis-associated-with-hemodialysis
#1
Faisal M Huq Ronny, George Kleinman, Paul James Kurtin, John Thomas Fallon
Dialysis-related amyloidosis predominantly occurs in osteo-articular structures and dialysis-related amyloid (DRA) substances also deposit in extra-articular tissues. Clinical manifestations of DRA include odynophagia, gastrointestinal hemorrhage, intestinal obstruction, kidney stones, myocardial dysfunction, and subcutaneous tumors. The pathological characteristics of DRA in the heart of hemodialysis patients have rarely been reported. We report the case of a 73-year-old female with a history of cerebral palsy and end-stage renal disease status post two failed renal transplants who had been on hemodialysis for 30 years...
January 2017: Autopsy & case reports
https://www.readbyqxmd.com/read/28468825/small-molecule-mediated-inhibition-of-%C3%AE-2-microglobulin-amyloid-fibril-formation
#2
Tyler M Marcinko, Jia Dong, Raquel LeBlanc, Kate V Daborowski, Richard W Vachet
In dialysis patients, β-2 microglobulin (β2m) can aggregate and eventually form amyloid fibrils in a condition known as dialysis-related amyloidosis, which deleteriously affects joint and bone function. Recently, several small molecules have been identified as potential inhibitors of β2m amyloid formation in vitro. Here, we investigate if these molecules are more broadly applicable inhibitors of β2m amyloid formation by studying their effect on Cu(II)-induced β2m amyloid formation. Using a variety of biophysical techniques, we also examine their inhibitory mechanisms...
May 3, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28455257/molecular-insights-into-the-inhibitory-mechanism-of-rifamycin-sv-against-%C3%AE-2-microglobulin-aggregation-a-molecular-dynamics-simulation-study
#3
Simranjeet Singh Narang, Suniba Shuaib, Bhupesh Goyal
Dialysis-related amyloidosis (DRA) is a severe condition characterized by the accumulation of amyloidogenic β2-microglobulin (β2m) protein around skeletal joints and bones. The small molecules that modulate β2m aggregation have been identified in vitro, however, the underlying inhibitory mechanism remain elusive. In the present study, molecular docking and molecular dynamics (MD) simulations were performed to elucidate the inhibitory mechanism of an antibiotic, rifamycin SV (C1) reported for its in vitro anti-aggregation activity against β2m...
April 25, 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/28359658/unrecognized-fibrinogen-a-%C3%AE-chain-amyloidosis-results-from%C3%A2-targeted-genetic-testing
#4
Isabel Tavares, João Paulo Oliveira, Ana Pinho, Luciana Moreira, Liliana Rocha, Josefina Santos, Joaquim Pinheiro, Paulo Pinho Costa, Luísa Lobato
BACKGROUND: Fibrinogen A α-chain (AFib) amyloidosis results from autosomal-dominant mutations in the gene encoding AFib (FGA). Patients with this disorder typically present with proteinuria. Isolated cases of AFib amyloidosis, carrying the FGA p.Glu545Val variant, were identified in the district of Braga, in northwest Portugal. This observation led us to hypothesize that this disorder might be an unrecognized cause of kidney disease in that region and prompted us to carry out targeted genetic testing for the p...
March 27, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28272335/proline-residues-as-switches-in-conformational-changes-leading-to-amyloid-fibril-formation
#5
Ajda Taler-Verčič, Samra Hasanbašić, Selma Berbić, Veronika Stoka, Dušan Turk, Eva Žerovnik
Here we discuss studies of the structure, folding, oligomerization and amyloid fibril formation of several proline mutants of human stefin B, which is a protein inhibitor of lysosomal cysteine cathepsins and a member of the cystatin family. The structurally important prolines in stefin B are responsible for the slow folding phases and facilitate domain swapping (Pro 74) and loop swapping (Pro 79). Moreover, our findings are compared to β₂-microglobulin, a protein involved in dialysis-related amyloidosis...
March 7, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28264226/dialysis-related-amyloidosis-is-it-gone-or-should-it-be
#6
EDITORIAL
Laura Labriola, Michel Jadoul
The prevalence and severity of dialysis-related amyloidosis (DRA) appear to have decreased significantly over the last two decades, although recent, large-scale epidemiological studies show that DRA continues to occur. Recent experimental findings have documented a direct cellular toxicity of β2microglobulin (β2m) fibrils but the mechanisms of β2m fibrillogenesis remain incompletely understood. Although a high plasma concentration of β2m is still considered as a prerequisite for developing DRA, other factors have been clearly incriminated such as older age at dialysis onset and longer dialysis vintage, or suspected effects such as proinflammatory effects of bioincompatible dialysis techniques...
May 2017: Seminars in Dialysis
https://www.readbyqxmd.com/read/28261626/influence-of-heparin-molecular-size-on-the-induction-of-c-terminal-unfolding-in-%C3%AE-2-microglobulin
#7
Kanon Fukasawa, Yuichiro Higashimoto, Yoshihiro Motomiya, Yoshinori Uji, Yukio Ando
Dialysis-related amyloidosis (DRA) is characterized by accumulation of amyloid β2- microglobulin (β2m) in the interstitial matrix. Matrix substances such as heparin have reportedly been strongly implicated in the pathogenesis of dialysis-related amyloidosis. In clinical setting of hemodialysis, two types of heparin, i.e., high and low molecular heparin (H.M.H. and L.M.H.) have been routinely used. Still commonly used is H.M.H., followed by L.M.H. preparations with distinct advantages. Here, we studied that the interaction of native and two amyloidogenic β2m variants: ΔN6β2m and D76N β2m with H...
December 2016: Molecular Biology Research Communications
https://www.readbyqxmd.com/read/28249361/heparin-induced-amyloid-fibrillation-of-%C3%AE-2-microglobulin-explained-by-solubility-and-a-supersaturation-dependent-conformational-phase-diagram
#8
Masatomo So, Yasuko Hata, Hironobu Naiki, Yuji Goto
Amyloid fibrils are fibrillar deposits of denatured proteins associated with amyloidosis and are formed by a nucleation and growth mechanism. We revisited an alternative and classical view of amyloid fibrillation: amyloid fibrils are crystal-like precipitates of denatured proteins formed above solubility upon breaking supersaturation. Various additives accelerate and then inhibit amyloid fibrillation in a concentration-dependent manner, suggesting that the combined effects of stabilizing and destabilizing forces affect fibrillation...
May 2017: Protein Science: a Publication of the Protein Society
https://www.readbyqxmd.com/read/28098102/evaluation-of-renal-lesions-and-clinicopathologic-correlation-in-rheumatoid-arthritis
#9
Periyasamy Muthukumar, Jeyachandran Dhanapriya, Natarajan Gopalakrishnan, Thanigachalam Dineshkumar, Ramanathan Sakthirajan, T Balasubramaniyan
The most common causes of renal disease in rheumatoid arthritis (RA) are glomerulonephritis (GN), amyloidosis, tubulo-interstitial nephritis, and drug toxicity. Our aim was to evaluate the clinicopathologic correlation of renal lesions and to assess the course and prognosis of renal disease in patients with RA. We conducted a prospective observational study in all adult patients with RA between July 2010 and June 2015. The total number of patients studied was 90, with a female:male ratio of 2.3:1. Mean follow-up duration was 30 ± 6...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27994478/dialysis-related-amyloidosis-challenges-and-solutions
#10
REVIEW
R Scarpioni, M Ricardi, V Albertazzi, S De Amicis, F Rastelli, L Zerbini
Amyloidosis refers to the extracellular tissue deposition of fibrils composed of low-molecular-weight subunits of a variety of proteins. These deposits may result in a wide range of clinical manifestations depending upon their type, location, and the amount of deposition. Dialysis-related amyloidosis is a serious complication of long-term dialysis therapy and is characterized by the deposition of amyloid fibrils, principally composed of β2 microglobulins (β2M), in the osteoarticular structures and viscera...
2016: International Journal of Nephrology and Renovascular Disease
https://www.readbyqxmd.com/read/27951570/biocompatibility-of-hemodiafilters
#11
Tadashi Tomo
BACKGROUND: Biocompatibility and the efficiency of solute removal are important considerations in blood purification therapy. Improvement of biocompatibility is expected to lead to the prevention of dialysis-related complications (e.g. amyloidosis, arteriosclerosis, and malnutrition) and to the delay of disease progression by alleviating microinflammation. SUMMARY: The biocompatibility of dialyzers is greatly influenced by the interaction between blood and the treatment materials, in which the chemical and physical characteristics of membrane materials play important roles...
2017: Contributions to Nephrology
https://www.readbyqxmd.com/read/27569937/comparative-analysis-of-outcomes-of-kidney-transplantation-in-patients-with-aa-amyloidosis-and-chronic-glomerulonephritis
#12
T Sahutoglu, K Atay, Y Caliskan, E Kara, H Yazici, A Turkmen
BACKGROUND: Amyloid A (AA) amyloidosis is a multisystemic, progressive, and severe disease. Renal involvement is a prominent feature of the disease, and the outcome of patients on dialysis is poor. We aimed to analyze the outcomes of kidney transplantation in patients with AA amyloidosis in comparison with chronic glomerulonephritis (CGN). METHODS: Charts of patients who underwent kidney transplantation between 1988 and 2012 were reviewed; 41 patients with AA amyloidosis were identified, and 41 age- and sex-matched control patients with chronic CGN were included...
July 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27491541/hemodynamic-deterioration-after-aortic-valve-replacement-in-a-patient-with-mixed-systemic-amyloidosis
#13
Tatsuya Seki, Atsuo Hattori, Toshihito Yoshida
We report a case of hemodynamic deterioration after aortic valve replacement in a patient with mixed systemic amyloidosis. A 77-year-old male with severe aortic valve stenosis and 19 years hemodialysis underwent aortic valve replacement. Postoperatively, the patient died of hemodynamic deterioration. Autopsy findings showed massive, whole-body edema and mixed systemic amyloidosis (dialysis-related and AA amyloidosis). Clinical and autopsy findings implied that hemodynamic deterioration was caused by increased vascular permeability...
August 4, 2016: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27467899/characterization-of-salt-induced-oligomerization-of-human-%C3%AE-2-microglobulin-at-low-ph
#14
Dominic Narang, Anubhuti Singh, Hema M Swasthi, Samrat Mukhopadhyay
Misfolding and amyloid aggregation of human β2-microglobulin (β2m) have been linked to dialysis-related amyloidosis. Previous studies have shown that in the presence of different salt concentrations and at pH 2.5, β2m assembles into aggregates with distinct morphologies. However, the structural and mechanistic details of the aggregation of β2m, giving rise to different morphologies, are poorly understood. In this work, we have extensively characterized the salt-induced oligomers of the acid-unfolded state of β2m using an array of biophysical tools including steady-state and time-resolved fluorescence, circular dichroism, dynamic light scattering, and atomic force microscopy imaging...
August 18, 2016: Journal of Physical Chemistry. B
https://www.readbyqxmd.com/read/27340120/renal-amyloid-a-amyloidosis-as-a-complication-of-hidradenitis-suppurativa
#15
Kristine D Schandorff, Iben M Miller, Dorrit Krustrup, Gregor B E Jemec, Peter Marckmann
Rheumatic disease is the dominant cause of amyloid A (AA) amyloidosis, but other chronic inflammatory diseases may have similar consequences. Hidradenitis suppurativa (HS) is a relatively common, but little known skin disease characterized by chronic inflammation. Here we present a case of chronic HS leading to biopsy-verified severe renal AA amyloidosis and dialysis dependency.
July 2016: Clinical Nephrology
https://www.readbyqxmd.com/read/27339443/a-clinical-staging-score-to-measure-the-severity-of-dialysis-related-amyloidosis
#16
Junichi Hoshino, Masahiro Kawada, Aya Imafuku, Koki Mise, Keiichi Sumida, Rikako Hiramatsu, Eiko Hasegawa, Noriko Hayami, Masayuki Yamanouchi, Tatsuya Suwabe, Naoki Sawa, Yoshifumi Ubara, Kenmei Takaichi
BACKGROUND: The ongoing effort to prevent dialysis-related amyloidosis (DRA) has been hampered by lack of any way to measure DRA's severity. Yet, such measurement is essential for assessing the effect of DRA treatment. Accordingly, we developed a scoring system focused on the physical manifestations of DRA. METHODS: Forty-four patients on maintenance hemodialysis with DRA, and 96 without it, were enrolled. The SF-36v2 Health Survey ascertained whether patients experienced general bodily pain and/or physical dysfunction with any attendant specific pain (dysfunction)...
June 23, 2016: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/27225299/stepwise-unfolding-of-human-%C3%AE-2-microglobulin-into-a-disordered-amyloidogenic-precursor-at-low-ph
#17
Dominic Narang, Anubhuti Singh, Samrat Mukhopadhyay
Amyloid fibril formation by human β2-microglobulin (β2m) is associated with dialysis-related amyloidosis. In order to understand the mechanism of protein misfolding, it is important to characterize the nature and properties of various intermediates formed during protein unfolding. In this work, we studied the effect of pH change on the unfolding of β2m using a range of spectroscopic readouts. In order to investigate the local structural changes, we created single tryptophan (W60 and W95) mutants of β2m...
January 2017: European Biophysics Journal: EBJ
https://www.readbyqxmd.com/read/27208169/the-role-of-fibrinogen-glycation-in-attr-evidence-for-chaperone-activity-loss-in-disease
#18
Daniel Fonseca, Samuel Gilberto, Cristina Ribeiro-Silva, Raquel Ribeiro, Inês Batista Guinote, Susana Saraiva, Ricardo A Gomes, Élia Mateus, Ana Viana, Eduardo Barroso, Ana Ponces Freire, Patrick Freire, Carlos Cordeiro, Gonçalo da Costa
Transthyretin amyloidosis (ATTR) belongs to a class of disorders caused by protein misfolding and aggregation. ATTR is a disabling disorder of autosomal dominant trait, where transthyretin (TTR) forms amyloid deposits in different organs, causing dysfunction of the peripheral nervous system. We previously discovered that amyloid fibrils from ATTR patients are glycated by methylglyoxal. Even though no consensus has been reached about the actual role of methylglyoxal-derived advanced glycation end-products in amyloid diseases, evidence collected so far points to a role for protein glycation in conformational abnormalities, being ubiquitously found in amyloid deposits in Alzheimer's disease, dialysis-related amyloidosis and Parkinson's diseases...
July 15, 2016: Biochemical Journal
https://www.readbyqxmd.com/read/27150430/rational-design-of-mutations-that-change-the-aggregation-rate-of-a-protein-while-maintaining-its-native-structure-and-stability
#19
Carlo Camilloni, Benedetta Maria Sala, Pietro Sormanni, Riccardo Porcari, Alessandra Corazza, Matteo De Rosa, Stefano Zanini, Alberto Barbiroli, Gennaro Esposito, Martino Bolognesi, Vittorio Bellotti, Michele Vendruscolo, Stefano Ricagno
A wide range of human diseases is associated with mutations that, destabilizing proteins native state, promote their aggregation. However, the mechanisms leading from folded to aggregated states are still incompletely understood. To investigate these mechanisms, we used a combination of NMR spectroscopy and molecular dynamics simulations to compare the native state dynamics of Beta-2 microglobulin (β2m), whose aggregation is associated with dialysis-related amyloidosis, and its aggregation-resistant mutant W60G...
2016: Scientific Reports
https://www.readbyqxmd.com/read/26757617/a-systematic-molecular-dynamics-approach-to-the-structural-characterization-of-amyloid-aggregation-propensity-of-%C3%AE-2-microglobulin-mutant-d76n
#20
P Chandrasekaran, R Rajasekaran
Beta-2 microglobulin (β2m) is an amyloidogenic protein belongs to the immunoglobulin superfamily, responsible for the dialysis-related amyloidosis (DRA). Misfolding of β2m is a prerequisite to the formation of systemic amyloidosis that has an effect on the structure and function of the affected organ. The aim of our present study is to intensively explore the structural characterization of amyloid aggregation propensity of recently identified natural mutation D76N by applying the classical molecular dynamics (MD) approach...
March 2016: Molecular BioSystems
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