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Amyloidosis

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https://www.readbyqxmd.com/read/28738073/the-n-terminal-region-of-serum-amyloid-a3-protein-activates-nf-%C3%AE%C2%BAb-and-up-regulates-muc2-mucin-mrna-expression-in-mouse-colonic-epithelial-cells
#1
Manami Tashiro, Ami Iwata, Marika Yamauchi, Kaori Shimizu, Ayaka Okada, Naotaka Ishiguro, Yasuo Inoshima
Serum amyloid A (SAA) is the major acute-phase protein and a precursor of amyloid A (AA) in AA amyloidosis in humans and animals. SAA isoforms have been identified in a wide variety of animals, such as SAA1, SAA2, SAA3, and SAA4 in mouse. Although the biological functions of SAA isoforms are not completely understood, recent studies have suggested that SAA3 plays a role in host defense. Expression of SAA3 is increased on the mouse colon surface in the presence of microbiota in vivo, and it increases mRNA expression of mucin 2 (MUC2) in murine colonic epithelial cells in vitro, which constitutes a protective mucus barrier in the intestinal tract...
2017: PloS One
https://www.readbyqxmd.com/read/28737773/the-incidence-of-atrial-fibrillation-among-patients-with-al-amyloidosis-undergoing-high-dose-melphalan-and-stem-cell-transplantation-experience-at-a-single-institution
#2
M Arun, D Brauneis, G Doros, A C Shelton, J M Sloan, K Quillen, F L Ruberg, V Sanchorawala, C Varga
No abstract text is available yet for this article.
July 24, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28737209/lactate-dehydrogenase-as-a-prognostic-marker-in-al-amyloidosis-expected-or-unexpected
#3
EDITORIAL
Chor S Chim
No abstract text is available yet for this article.
July 24, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28736891/heat-induced-native-dimerization-prevents-amyloid-formation-by-variable-domain-from-immunoglobulin-light-chain-rei
#4
Marina Nawata, Hirotaka Tsutsumi, Yuta Kobayashi, Satoru Unzai, Shouhei Mine, Tsutomu Nakamura, Koichi Uegaki, Hironari Kamikubo, Mikio Kataoka, Daizo Hamada
Amyloid light chain (AL) amyloidosis is a protein-misfolding disease characterized by accumulation of immunoglobulin light chains (LCs) into amyloid fibrils. Dimerization of full length or variable domain (VL ) of LC serves to stabilize the native state and prevent the formation of amyloid fibrils. We here analyzed the thermodynamic properties of dimerization and unfolding reactions by nonamyloidogenic VL from REI LC or its monomeric Y96K mutant using sedimentation velocity and circular dichroism. The data indicate that the equilibrium shifts to native dimerization for wild type REI VL by elevating temperature due to the negative enthalpy change for dimer dissociation (-81...
July 24, 2017: FEBS Journal
https://www.readbyqxmd.com/read/28734653/amyloid-%C3%AE-concentrations-and-stable-isotope-labeling-kinetics-of-human-plasma-specific-to-central-nervous-system-amyloidosis
#5
Vitaliy Ovod, Kara N Ramsey, Kwasi G Mawuenyega, Jim G Bollinger, Terry Hicks, Theresa Schneider, Melissa Sullivan, Katrina Paumier, David M Holtzman, John C Morris, Tammie Benzinger, Anne M Fagan, Bruce W Patterson, Randall J Bateman
INTRODUCTION: Cerebrospinal fluid analysis and other measurements of amyloidosis, such as amyloid-binding positron emission tomography studies, are limited by cost and availability. There is a need for a more practical amyloid β (Aβ) biomarker for central nervous system amyloid deposition. METHODS: We adapted our previously reported stable isotope labeling kinetics protocol to analyze the turnover kinetics and concentrations of Aβ38, Aβ40, and Aβ42 in human plasma...
July 11, 2017: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/28733641/influence-of-c-terminal-truncation-of-murine-serum-amyloid-a-on-fibril-structure
#6
Matthies Rennegarbe, Inga Lenter, Angelika Schierhorn, Romy Sawilla, Christian Haupt
Amyloid A (AA) amyloidosis is a systemic protein misfolding disease affecting humans and other vertebrates. While the protein precursor in humans and mice is the acute-phase reactant serum amyloid A (SAA) 1.1, the deposited fibrils consist mainly of C-terminally truncated SAA fragments, termed AA proteins. For yet unknown reasons, phenotypic variations in the AA amyloid distribution pattern are clearly associated with specific AA proteins. Here we describe a bacterial expression system and chromatographic strategies to obtain significant amounts of C-terminally truncated fragments of murine SAA1...
July 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28729244/apoptosis-repressor-with-caspase-recruitment-domain-ameliorates-amyloid-induced-%C3%AE-cell-apoptosis-and-jnk-pathway-activation
#7
Andrew T Templin, Tanya Samarasekera, Daniel T Meier, Meghan F Hogan, Mahnaz Mellati, Michael T Crow, Richard N Kitsis, Sakeneh Zraika, Rebecca L Hull, Steven E Kahn
Islet amyloid is present in more than 90% of individuals with type 2 diabetes, where it contributes to β-cell apoptosis and insufficient insulin secretion. Apoptosis repressor with caspase recruitment domain (ARC) binds and inactivates components of the intrinsic and extrinsic apoptosis pathways, and was recently found to be expressed in islet β cells. Using a human islet amyloid polypeptide transgenic mouse model of islet amyloidosis, we show ARC knockdown increases amyloid-induced β-cell apoptosis and loss, while ARC overexpression decreases amyloid-induced apoptosis, thus preserving β cells...
July 20, 2017: Diabetes
https://www.readbyqxmd.com/read/28729192/butyrylcholinesterase-knockout-reduces-fibrillar-%C3%AE-amyloid-and-conserves-18-fdg-retention-in-5xfad-mouse-model-of-alzheimer-s-disease
#8
D R DeBay, G A Reid, I R Macdonald, G Mawko, S Burrell, E Martin, C V Bowen, S Darvesh
Alzheimer's disease (AD) is the most common neurodegenerative disorder causing dementia. One hallmark of the AD brain is the deposition of β-amyloid (Aβ) plaques. AD is also a state of cholinergic dysfunction and butyrylcholinesterase (BChE) associates with Aβ pathology. A transgenic mouse (5XFAD) is an aggressive amyloidosis model, producing Aβ plaques of which BChE also associates. A derived strain (5XFAD/BChE-KO), with the BChE gene knocked out, has significantly lower fibrillar Aβ than 5XFAD mice at the same age...
July 17, 2017: Brain Research
https://www.readbyqxmd.com/read/28729034/prolonged-renal-survival-in-light-chain-amyloidosis-speed-and-magnitude-of-light-chain-reduction-is-the-crucial-factor
#9
Tamer Rezk, Helen J Lachmann, Marianna Fontana, Sajitha Sachchithanantham, Shameem Mahmood, Aviva Petrie, Carol J Whelan, Jennifer H Pinney, Darren Foard, Thirusha Lane, Taryn Youngstein, Ashutosh D Wechalekar, Paul Bass, Philip N Hawkins, Julian D Gillmore
Renal involvement causing progressive chronic kidney disease (CKD) is present in 70% of patients with systemic Ig light-chain (AL) amyloidosis at diagnosis. Chemotherapy that substantially suppresses free light chain production is associated with improved patient survival, but its benefit in delaying the onset of renal replacement therapy among patients who present with established advanced CKD has not been studied. To evaluate this, we studied 1000 patients enrolled in the prospective UK AL amyloidosis chemotherapy study (ALchemy)...
July 17, 2017: Kidney International
https://www.readbyqxmd.com/read/28728693/broadening-the-phenotypic-spectrum-and%C3%A2-the-diagnostic-needs-of-ttr-related-cardiac-amyloidosis
#10
EDITORIAL
Claudio Rapezzi, Anna Laura Tinuper, Massimiliano Lorenzini
No abstract text is available yet for this article.
July 25, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28728692/magnetic-resonance-in-transthyretin%C3%A2-cardiac-amyloidosis
#11
Ana Martinez-Naharro, Thomas A Treibel, Amna Abdel-Gadir, Heerajnarain Bulluck, Giulia Zumbo, Daniel S Knight, Tushar Kotecha, Rohin Francis, David F Hutt, Tamer Rezk, Stefania Rosmini, Candida C Quarta, Carol J Whelan, Peter Kellman, Julian D Gillmore, James C Moon, Philip N Hawkins, Marianna Fontana
BACKGROUND: Cardiac transthyretin amyloidosis (ATTR) is an increasingly recognized cause of heart failure. Cardiac magnetic resonance (CMR), with late gadolinium enhancement (LGE) and T1 mapping, is emerging as a reference standard for diagnosis and characterization of cardiac amyloidosis. OBJECTIVES: The authors used CMR with extracellular volume fraction (ECV) measurement to characterize cardiac involvement in relation to outcome in ATTR. METHODS: Subjects comprised 263 patients with cardiac ATTR corroborated by grade 2 to 3 (99m)Tc-DPD ((99m)Tc-3,3-diphosphono-1,2-propanodicarboxylic acid) cardiac uptake, 17 with suspected cardiac ATTR (grade 1 (99m)Tc-DPD), and 12 asymptomatic individuals with amyloidogenic transthyretin (TTR) mutations...
July 25, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28725274/news-in-al-amyloidosis-ash-2016-a%C3%A2-rapidly-evolving-field-of-investigation
#12
REVIEW
Hermine Agis
Amyloidosis is a rare but life-threatening protein misfolding disease. The early diagnosis and enrollment of patients into multicentre trials is of great importance, as is the need for intensive collaboration between multiple medical departments and experienced specialists. In the following review, the most interesting abstracts from the annual American Society of Hematology (ASH) meeting in 2016 are presented. The topics include the limitations of established biomarkers in risk assessment and response evaluation, the introduction of a new biomarker, the comparison of different treatment sequences and the efficacy of a multiple drug regimen in light-chain (AL) amyloidosis...
2017: Memo
https://www.readbyqxmd.com/read/28723285/case-244-systemic-amyloidosis-a-complication-of-waldenstr%C3%A3-m-macroglobulinemia
#13
Maja Hrabak-Paar, Marko Kralik
History A 68-year-old man was admitted to the hospital for work-up because of generalized fatigue, anorexia, chronic diarrhea, and weight loss. Laboratory work-up revealed an erythrocyte sedimentation rate of 58 mm/h (reference range, 3-23 mm/h), a hemoglobin level of 14.1 g/dL (reference range, 13.8-17.5 g/dL), a leukocyte count of 8.1 × 10(9)/L (reference range, [3.4-9.7] × 10(9)/L), a platelet count of 223 × 10(9)/L (reference range, [158-424] × 10(9)/L), an alkaline phosphatase level of 85 U/L (1.42 μkat/L) (normal level, <142 U/L [2...
August 2017: Radiology
https://www.readbyqxmd.com/read/28721361/role-of-endoplasmic-reticulum-stress-in-learning-and-memory-impairment-and-alzheimer-s-disease-like-neuropathology-in-the-ps19-and-app-swe-mouse-models-of-tauopathy-and-amyloidosis
#14
Denise Isabelle Briggs, Erwin Defensor, Pooneh Memar Ardestani, Bitna Yi, Michelle Halpain, Guy Seabrook, Mehrdad Shamloo
Emerging evidence suggests that endoplasmic reticulum (ER) stress may be involved in the pathogenesis of Alzheimer's disease (AD). Recently, pharmacological modulation of the eukaryotic translation initiation factor-2 (eIF2α) pathway was achieved using an integrated stress response inhibitor (ISRIB). While members of this signaling cascade have been suggested as potential therapeutic targets for neurodegeneration, the biological significance of this pathway has not been comprehensively assessed in animal models of AD...
July 2017: ENeuro
https://www.readbyqxmd.com/read/28719236/applying-an-artificial-neural-network-model-for-developing-a-severity-score-for-patients-with-hereditary-amyloid-polyneuropathy
#15
Shenia Novis, Felipe Machado, Victor B Costa, Debora Foguel, Marcia W Cruz, José Manoel de Seixas
Hereditary (familial) amyloid polyneuropathy (FAP) is a systemic disease that includes a sensorimotor polyneuropathy related to transthyretin (TTR) mutations. So far, a scale designed to classify the severity of this disease has not yet been validated. This work proposes the implementation of an artificial neural network (ANN) in order to develop a severity scale for monitoring the disease progression in FAP patients. In order to achieve this goal, relevant symptoms and laboratory findings were collected from 98 Brazilian patients included in THAOS - the Transthyretin Amyloidosis Outcomes Survey...
July 18, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28719235/artificial-neural-network-modelling-of-attr-amyloidosis-is-now-the-time
#16
John L Berk
No abstract text is available yet for this article.
July 18, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28716239/acquired-transthyretin-amyloidosis-after-domino-liver-transplant-phenotypic-correlation-implication-of-liver-retransplantation
#17
Eli Muchtar, Martha Grogan, Surendra Dasari, Paul J Kurtin, Morie A Gertz
Reports of acquired ATTRm in domino liver transplant (DLT) recipients are infrequent and the approach to management is unknown. We describe two patients, an inherited ATTRm patient who underwent liver transplant and his liver recipient who developed acquired ATTRm 8years following transplantation. The clinical manifestations of amyloidosis were similar between patients and consisted of peripheral neuropathy, diarrhea and weight loss. Following liver transplantation, the DLT donor went into remission for 4years, but eventually progressed and died...
August 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28711693/amyloidosis-an-unusual-cause-of-intestinal-pseudo-obstruction
#18
Jie Wu, Yongjun Wang, Chunlian Wang
No abstract text is available yet for this article.
July 12, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28705053/cardiac-amyloidosis-pathogenesis-clinical-context-diagnosis-and-management-options
#19
Hassan Alkhawam, Darshan Patel, James Nguyen, Saumya Mariam Easaw, Mohammed Al-Sadawi, Umer Syed, Feras Zaiem, Maher Homsi, Timothy J Vittorio
Amyloidosis covers a group of disorders that can manifest in virtually any organ system in the body and is thought to be secondary to misfolding of extracellular proteins with subsequent deposition in tissues. The precursor protein that is produced in excess defines the specific amyloid type. This requires histopathological confirmation using Congo-red dye with its characteristic demonstration of green birefringence under cross-polarized light. There are three main types of amyloidosis associated with cardiac involvement: light-chain (AL), familial or senile (ATTR), and secondary (AA) amyloidosis...
July 14, 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28704493/evaluating-the-effect-of-mutations-and-ligand-binding-on-transthyretin-homotetramer-dynamics
#20
Tadeo E Saldaño, Giuseppe Zanotti, Gustavo Parisi, Sebastian Fernandez-Alberti
Native transthyretin (TTR) homotetramer dissociation is the first step of the fibrils formation process in amyloid disease. A large number of specific point mutations that destabilize TTR quaternary structure have shown pro-amyloidogenic effects. Besides, several compounds have been proposed as drugs in the therapy of TTR amyloidosis due to their TTR tetramer binding affinities, and therefore, contribution to its integrity. In the present paper we have explored key positions sustaining TTR tetramer dynamical stability...
2017: PloS One
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