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Amyloid: the International Journal of Experimental and Clinical Investigation

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https://www.readbyqxmd.com/read/28081656/clinicopathological-and-biochemical-findings-of-thyroid-amyloid-in-hereditary-transthyretin-amyloidosis-with-and-without-liver-transplantation
#1
Guannan Huang, Mitsuharu Ueda, Masayoshi Tasaki, Taro Yamashita, Yohei Misumi, Teruaki Masuda, Genki Suenaga, Yasuteru Inoue, Yumiko Kinoshita, Sayaka Matsumoto, Mayumi Mizukami, Yukimoto Tsuda, Toshiya Nomura, Konen Obayashi, Yukio Ando
Hereditary transthyretin (TTR) amyloidosis is a fatal disease causing systemic organ dysfunctions. Histopathological studies revealed that thyroid glands are major target tissues. However, details about thyroid functions remain to be fully elucidated in this disease. For patient treatment, liver transplantation (LT) reportedly prolongs patient survival, but thyroid gland function after LT still remains poorly understood. In this study, we investigated the thyroid functions in 101 patients with hereditary TTR amyloidosis and the effects of LT on thyroid functions in those patients...
January 13, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28081655/marked-biochemical-difference-in-amyloid-proportion-between-intra-and-extraocular-tissues-in-a-liver-transplanted-patient-with-hereditary-attr-amyloidosis
#2
Tsuneaki Yoshinaga, Masahide Yazaki, Fuyuki Kametani, Yoshiki Sekijima, Yasuhiro Iesato, Teruyoshi Miyahara, Ayako Tsuchiya-Suzuki, Kenji Sano, Keiichi Higuchi, Shu-Ichi Ikeda
In order to elucidate the pathomechanism of ocular amyloid formation in a liver-transplanted patient with hereditary ATTR amyloidosis, we investigated detailed biochemical features of ocular amyloid. The patient was a 49-year-old woman with V30M transthyretin (TTR) variant (p.TTRV50M), who underwent ophthalmectomy due to corneal rupture 10 years after liver transplantation (LT). The amyloid was selectively isolated from several portions in intra- and extraocular tissues using a laser microdissection (LMD) system and analyzed by liquid chromatography-tandem mass spectrometry to determine the composition percentage of wild-type and variant TTR in the isolated amyloid...
January 13, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28042712/structure-of-serum-amyloid-a-suggests-a-mechanism-for-selective-lipoprotein-binding-and-functions-saa-as-a-hub-in-macromolecular-interaction-networks
#3
Nicholas M Frame, Olga Gursky
No abstract text is available yet for this article.
December 31, 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28042708/structural-stability-and-local-dynamics-in-disease-causing-mutants-of-human-apolipoprotein-a-i-what-makes-the-protein-amyloidogenic
#4
Madhurima Das, Christopher J Wilson, Xiaohu Mei, Thomas Wales, John R Engen, Olga Gursky
No abstract text is available yet for this article.
December 31, 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28042702/effect-of-doxycycline-and-ursodeoxycholic-acid-on-transthyretin-amyloidosis
#5
Jonas Wixner, Björn Pilebro, Hans-Erik Lundgren, Malin Olsson, Intissar Anan
No abstract text is available yet for this article.
December 31, 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28019722/immunotherapeutic-clearance-of-systemic-amyloid-deposits-by-antibodies-to-serum-amyloid-p-component
#6
Mark B Pepys
No abstract text is available yet for this article.
December 26, 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28019720/solid-and-solution-state-nuclear-magnetic-resonance-spectroscopic-studies-on-antibody-light-chain-amyloid-formation-and-interactions-with-epigallocatechin-gallate
#7
Manuel Hora, Martin Carballo Pacheco, Benedikt Weber, Johannes Buchner, Birgit Strodel, Bernd Reif
No abstract text is available yet for this article.
December 26, 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27884064/amyloid-fibril-proteins-and-amyloidosis-chemical-identification-and-clinical-classification-international-society-of-amyloidosis-2016-nomenclature-guidelines
#8
Jean D Sipe, Merrill D Benson, Joel N Buxbaum, Shu-Ichi Ikeda, Giampaolo Merlini, Maria J M Saraiva, Per Westermark
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, to assess and formulate recommendations for nomenclature for amyloid fibril proteins and the clinical classification of the amyloidoses. An amyloid fibril must exhibit affinity for Congo red and with green, yellow or orange birefringence when the Congo red-stained deposits are viewed with polarized light. While congophilia and birefringence remain the gold standard for demonstration of amyloid deposits, new staining and imaging techniques are proving useful...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27884058/efficiency-of-silencing-rna-for-removal-of-transthyretin-v30m-in-a-ttr-leptomeningeal-animal-model
#9
Paula Gonçalves, Helena Martins, Susete Costelha, Luis F Maia, Maria Joao Saraiva
Some TTR mutants target the central nervous system (CNS). Familial amyloid polyneuropathy (FAP) with leptomeningeal involvement has been described in 9% of transthyretin (TTR) mutations and in valine for methionine at position 30 (V30M) patients. These individuals present dementia, ataxia, brain hemorrhages and focal neurological episodes (FNEs). FNEs occurred also in V30M FAP patients with longer disease duration, who have undergone liver transplant to remove the source of plasma mutant TTR as a form of treatment...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27879149/increasing-amount-of-amyloid-are-associated-with-the-severity-of-clinical-features-in-hereditary-gelsolin-agel-amyloidosis
#10
Tiia Pihlamaa, Sinikka Suominen, Sari Kiuru-Enari, Maarit Tanskanen
BACKGROUND: Patients with hereditary gelsolin (AGel) amyloidosis (HGA) present with hanging skin (cutis laxa) and bilateral cranial neuropathy, and require symptomatic plastic surgery. Our clinical observation of tissue fragility prompted us to design a prospective study. METHODS: Twenty-nine patients with HGA undergoing surgery were interviewed and clinically examined. The height and thickness of skin folds in standard anatomical localizations were measured. The presence and distribution of amyloid in skin samples were analyzed using Congo red staining and immunohistochemistry using antibodies against gelsolin amyloid (AGel) subunit...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27879147/pre-transplantation-novel-agent-induction-predicts-progression-free-survival-for-patients-with-immunoglobulin-light-chain-amyloidosis-undergoing-high-dose-melphalan-and-autologous-stem-cell-transplantation
#11
Andrew J Cowan, Zandra K Klippel, Philip A Stevenson, Teresa S Hyun, Sherilyn Tuazon, Pamela S Becker, Damian J Green, Leona A Holmberg, David G Coffey, Ajay K Gopal, Edward N Libby
INTRODUCTION: High-dose melphalan and autologous stem cell transplantation (HDM/SCT) is an effective treatment modality for immunoglobulin light-chain (AL) amyloidosis; however, its application remains restricted to patients with good performance status and limited organ involvement. In recent years, the paradigm for AL amyloidosis has changed with the introduction of novel agents such as immunomodulatory drugs (IMiDs) and proteasome inhibitors (PIs). We hypothesized that use of novel agent induction regimens has improved outcomes for patients with AL amyloidosis undergoing HDM/SCT at our center...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27809600/plasma-hepatocyte-growth-factor-is-a-novel-marker-of-al-cardiac-amyloidosis
#12
Kristopher J Swiger, Eitan A Friedman, Evan L Brittain, Kelsey A Tomasek, Shi Huang, Yan R Su, Douglas B Sawyer, Daniel J Lenihan
BACKGROUND: Cardiac amyloidosis is an infiltrative cardiomyopathy that is challenging to diagnose. We hypothesized that the novel biomarkers hepatocyte growth factor (HGF), galectin-3 (GAL-3), interleukin-6 (IL-6), and vascular endothelial growth factor (VEGF) would be elevated in cardiac amyloidosis and may be able to discriminate from non-cardiac systemic amyloidosis or other cardiomyopathies with similar clinical or morphologic characteristics. METHODS: Patients were selected from the Vanderbilt Main Heart Registry according to the following groups: (1) amyloid light-chain (AL) cardiac amyloidosis (n = 26); (2) transthyretin (ATTR) cardiac amyloidosis (n = 7); (3) left ventricular hypertrophy (LVH) (n = 45); (4) systolic heart failure (n = 42); and (5) non-cardiac systemic amyloidosis (n = 7)...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27791396/citrullination-and-deamidation-affect-aggregation-properties-of-amyloid-%C3%AE-proteins
#13
Dai Osaki, Hirotsugu Hiramatsu
Citrullination and deamidation, which are aging-related posttranslational modifications, increase the number of negative charges on amyloid β-protein (Aβ) at neutral pH. We investigated the effects of these modifications on the fibrillation properties of Aβ. The Arg5→Cit modification of Aβ1-40 did not affect the fibrillation rate, and brought β-sheet structures unlike that in the Aβ1-40 fibril. The Asn27→Asp modification of Aβ1-40 stopped the fibrillation and induced the formation of aggregates that involved an anti-parallel β-sheet...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27791393/long-term-follow-up-on-tocilizumab-treatment-of-aa-amyloidosis-secondary-to-polyarteritis-nodosa
#14
Jaka Ostrovršnik, Alojzija Hočevar, Boris Lestan, Snežna Sodin Šemrl, Katja Lakota, Matija Tomšič
No abstract text is available yet for this article.
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27748624/tafamidis-in-hereditary-attr-amyloidosis-our-experience-on-monitoring-the-ocular-manifestations
#15
Inês Casal, Sílvia Monteiro, João Melo Beirão
No abstract text is available yet for this article.
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27682970/capillary-electrophoresis-immunosubtraction-as-a-better-alternative-to-immunofixation-for-detecting-and-immunotyping-serum-monoclonal-proteins-in-patients-with-immunoglobulin-light-chain-al-amyloidosis
#16
Kanji Miyazaki, Kenshi Suzuki
Capillary electrophoresis/immunosubtraction (CE/IS) is a simple method for detecting and immunotyping serum or urine monoclonal proteins. To our knowledge, there are no previous reports of the use of CE/IS for characterizing patients with Immunoglobulin light chain (AL) amyloidosis, and there are no convincing data available to compare CE/IS with serum immunofixation electrophoresis (IFE) and free light chain (FLC) assay. The aim of this study was to evaluate the clinical utility of CE/IS in patients with AL amyloidosis as a diagnostic accuracy study...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27677679/immunoglobulin-heavy-light-chain-test-quantifies-clonal-disease-in-patients-with-al-amyloidosis-and-normal-serum-free-light-chain-ratio
#17
Tatiana Prokaeva, Brian Spencer, Fangui Sun, Richard M O'Hara, David C Seldin, Lawreen H Connors, Vaishali Sanchorawala
BACKGROUND: Serum and urine immunofixation electrophoreses (SIFE/UIFE) are used for clonal detection in plasma cell dyscrasias, while serum free light chain (sFLC) testing provides quantitation of clonal disease. Up to 20% of patients with light chain (AL) amyloidosis may present with normal FLC ratio (FLCr). METHODS: We assessed the diagnostic, quantitative and prognostic potential of serum heavy light chain ratio (HLCr) in 199 untreated patients at initial evaluation...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27647161/identification-of-prognostic-markers-in-transthyretin-and-al-cardiac-amyloidosis
#18
MULTICENTER STUDY
Thibaud Damy, Arnaud Jaccard, Aziz Guellich, David Lavergne, Arnault Galat, Jean-François Deux, Luc Hittinger, Jehan Dupuis, Valérie Frenkel, Charlotte Rigaud, Violaine Plante-Bordeneuve, Diane Bodez, Dania Mohty
BACKGROUND: The prognosis of amyloidosis is known to depend heavily on cardiac function and may be improved by identifying patients at highest risk for adverse cardiac events. AIMS: Identify predictors of mortality in patients with cardiac light-chain amyloidosis (AL), hereditary transthyretin amyloidosis (m-TTR), or wild-type transthyretin amyloidosis (WT-TTR) to prompt physician to refer these patients to dedicated centers. METHODS AND RESULTS: Observational study...
September 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27646980/hereditary-amyloidosis-with-cardiomyopathy-caused-by-the-novel-variant-transthyretin-a36d
#19
Taro Yamashita, Mitsuharu Ueda, Nobuyuki Saga, Kiyonori Nanto, Masayoshi Tasaki, Teruaki Masuda, Yohei Misumi, Seitaro Oda, Akiko Fujimoto, Tomoko Amano, Kotaro Takamatsu, Satoshi Yamashita, Konen Obayashi, Hirotaka Matsui, Yukio Ando
No abstract text is available yet for this article.
September 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27494299/early-intervention-with-tafamidis-provides-long-term-5-5-year-delay-of-neurologic-progression-in-transthyretin-hereditary-amyloid-polyneuropathy
#20
RANDOMIZED CONTROLLED TRIAL
Márcia Waddington Cruz, Leslie Amass, Denis Keohane, Jeffrey Schwartz, Huihua Li, Balarama Gundapaneni
: Transthyretin hereditary amyloid polyneuropathy, also traditionally known as transthyretin familial amyloid polyneuropathy (ATTR-FAP), is a rare, relentless, fatal hereditary disorder. Tafamidis, an oral, non-NSAID, highly specific transthyretin stabilizer, demonstrated safety and efficacy in slowing neuropathy progression in early-stage ATTRV30M-FAP in a 1.5-year, randomized, double-blind, placebo-controlled trial, and 1-year open-label extension study, with a second long-term open-label extension study ongoing...
September 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
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