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https://www.readbyqxmd.com/read/28326625/alantolactone-and-isoalantolactone-prevent-amyloid-%C3%AE-25-35-induced-toxicity-in-mouse-cortical-neurons-and-scopolamine-induced-cognitive-impairment-in-mice
#1
Ji Yeon Seo, Soon Sung Lim, Jiyoung Kim, Ki Won Lee, Jong-Sang Kim
Given the evidence for detoxifying/antioxidant enzyme-inducing activities by alantolactone (AL) and isoalantolactone (IAL), the purpose of this study was to investigate the effects of AL and IAL on Aβ25-35 -induced cell death in mouse cortical neuron cells and to determine their effects on scopolamine-induced amnesia in mice. Our data demonstrated that both compounds effectively attenuated the cytotoxicity of Aβ25-35 (10 μM) in neuronal cells derived from the mouse cerebral cortex. It was also found that the production of intracellular reactive oxygen species, including superoxide anion induced by Aβ25-35 , was inhibited...
March 21, 2017: Phytotherapy Research: PTR
https://www.readbyqxmd.com/read/28295502/assessment-of-renal-response-with-urinary-exosomes-in-patients-with-al-amyloidosis-a-proof-of-concept
#2
Marina Ramirez-Alvarado, David R Barnidge, David L Murray, Angela Dispenzieri, Marta Marin-Argany, Christopher J Dick, Shawna A Cooper, Samih H Nasr, Christopher J Ward, Surendra Dasari, Víctor H Jiménez-Zepeda, Nelson Leung
Immunoglobulin light chain (AL) amyloidosis is a fatal complication of B-cell proliferation secondary to deposition of amyloid fibrils in various organs. Urinary exosomes (UEX) are the smallest of the microvesicles excreted in the urine. Previously, we found UEX of patients with AL amyloidosis contained immunoglobulin light chain (LC) oligomers that patients with multiple myeloma did not have. To further explore the role of the LC oligomers, UEX was isolated from an AL amyloidosis patient with progressive renal disease despite achieving a complete response...
March 10, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28293126/the-risk-of-renal-disease-is-increased-in-lambda-myeloma-with-bone-marrow-amyloid-deposits
#3
Piotr Kozlowski, Scott Montgomery, Rahel Befekadu, Victoria Hahn-Strömberg
BACKGROUND: Light chain amyloidosis (AL) is a rare deposition disease and is present in 10-15% of patients with myeloma (MM). In contrast to symptomatic AL in MM, presence of bone marrow (BM) amyloid deposits (AD) in MM is not connected to kidney damage. Renal AD but not BM-AD occur mostly in MM with lambda paraprotein (lambda MM). METHODS: We investigated amyloid presence in BM clots taken at diagnosis in 84 patients with symptomatic MM and compared disease characteristics in MM with kappa paraprotein (kappa MM)/lambda MM with and without BM-AD...
2017: Journal of Blood Medicine
https://www.readbyqxmd.com/read/28290973/total-artificial-heart-implantation-as-a-bridge-to-heart-transplantation-in-an-active-duty-service-member-with-amyloid-cardiomyopathy
#4
Michael S Scully, Dylan E Wessman, James M McKee, Gregory M Francisco, Keshav R Nayak, Jon A Kobashigawa
INTRODUCTION: Cardiac involvement by light-chain (AL) amyloid occurs in up to 50% of patients with primary AL amyloidosis. The prognosis of amyloid heart disease is poor with 1-year survival rates of 35 to 40%. Historically, heart transplantation was considered controversial for patients with AL amyloid cardiomyopathy (CM) given the systemic nature of the disease and poor survival. We present a case report of an active duty service member diagnosed with advanced cardiac amyloid who underwent total artificial heart transplant as a bridge to heart transplant and eventual autologous stem cell transplant...
March 2017: Military Medicine
https://www.readbyqxmd.com/read/28286788/primary-hepatic-amyloidosis-presenting-as-acute-on-chronic-liver-failure
#5
Madhumita Premkumar, Devaraja Rangegowda, Tanmay Vyas, Anand Kulkarni, Shrruti Grover, Rakhi Mahiwall, Shiv Kumar Sarin
Systemic amyloidosis of amyloid light chain associated protein (AL), also called primary amyloidosis, frequently involves the liver, but rarely causes clinically apparent liver disease. The more common presentation is with acute renal failure. Hepatomegaly and mild elevation of alkaline phosphatase are the most common clinical and biochemical findings, respectively. We report a case of systemic amyloidosis of AL that clinically presented as acute-on-chronic liver failure and resulted in a fatal clinical course in a 56-year-old man...
2017: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28285172/multifunctional-luv-liposomes-decorated-for-bbb-and-amyloid-targeting-b-in-vivo-brain-targeting-potential-in-wild-type-and-app-ps1-mice
#6
Konstantina Papadia, Anastasios D Giannou, Eleni Markoutsa, Christian Bigot, Greejte Vanhoute, Spyridon Mourtas, Annemie Van der Linded, Georgios T Stathopoulos, Sophia G Antimisiaris
Multifunctional liposomes (mf-LIPs) having a curcumin-lipid ligand (to target amyloids) together with two ligands to target the transferrin, and the low-density apolipoprotein receptor of the blood-brain-barrier (BBB) on their surface, were previously studied (in vitro) as potential theranostic systems for Alzheimer's disease (AD) (Papadia et al., 2017, Eur. J. Pharm. Sciences; 101:140-148). Herein, the targeting potential of mf-LIPs was compared to that of BBB-LIPs (liposomes having only the two BBB-specific ligands) in FVB mice (normal), as well as in double transgenic mice (APP/PS1) and their corresponding littermates (WT), by live-animal (in vivo) and explanted organ (ex vivo) imaging...
March 8, 2017: European Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/28281017/heart-transplantation-in-cardiac-amyloidosis
#7
REVIEW
Matthew Sousa, Gregory Monohan, Navin Rajagopalan, Alla Grigorian, Maya Guglin
"Cardiac amyloidosis" is the term commonly used to reflect the deposition of abnormal protein amyloid in the heart. This process can result from several different forms, most commonly from light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis, which in turn can represent wild-type (ATTRwt) or genetic form. Regardless of the origin, cardiac involvement is usually associated with poor prognosis, especially in AL amyloidosis. Although several treatment options, including chemotherapy, exist for different forms of the disease, cardiac transplantation is increasingly considered...
March 9, 2017: Heart Failure Reviews
https://www.readbyqxmd.com/read/28279360/testing-the-amyloid-hypothesis-with-a-humanized-ad-mouse-model
#8
Michelle K Cahill, Eric J Huang
In this issue of Neuron, Espuny-Camacho et al. (2017) generate a humanized Alzheimer's disease (AD) model that reveals species-specific vulnerability of human neurons to AD pathology. This model provides key insights for disease mechanism and therapeutic discovery for AD.
March 8, 2017: Neuron
https://www.readbyqxmd.com/read/28260291/-andersson-lesion-in-ankylosing-spondylitis-a-clinical-study-of-14-cases
#9
Y H Li, X Y Zhang, Q H Li, D H Zheng, L Dai
Objective: To investigate the clinical characteristics of ankylosing spondylitis (AS) combined Andersson lesion (AL). Methods: The clinical data of patients who were diagnosed as AS combined AL at Sun Yat-sen Memorial Hospital between January 2012 and December 2015 were reviewed retrospectively.SPSS 20.0 software was used for statistical analysis.Data of normal distribution was expressed by x±s (standard deviation) and that of abnormal distribution by median and range. Results: Fourteen patients were enrolled...
February 21, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28256403/the-utility-of-99m-tc-dpd-scintigraphy-in-the-diagnosis-of-cardiac-amyloidosis-an-australian-experience
#10
Peter T Moore, Matthew K Burrage, Emily Mackenzie, W Philip Law, Dariusz Korczyk, Peter Mollee
BACKGROUND: The uptake of bone-seeking radiotracers in the amyloid heart is well recognised. 99(m)Tc-DPD has been shown to be highly sensitive for cardiac transthyretin (ATTR) amyloid in an overseas population, but is not registered for use in Australia. We explored its utility as a diagnostic tool within our population. METHODS: Patients diagnosed with AL and ATTR (wild-type and inherited) cardiac amyloidosis were prospectively recruited from the Princess Alexandra Hospital Amyloidosis Centre...
February 16, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28202864/clinical-presentation-of-tubulointerstitial-nephritis-caused-by-amyloid-light-chain-amyloidosis-in-a-patient-with-sj%C3%A3-gren-s-syndrome
#11
Reiko Inoue, Yoshihide Fujigaki, Kana Kobayashi, Yoshifuru Tamura, Tatsuru Ota, Shigeru Shibata, Tsuyoshi Ishida, Fukuo Kondo, Yutaka Yamaguchi, Shunya Uchida
We report a 70-year-old woman with Sjögren's syndrome who had severe renal dysfunction with mild proteinuria and elevated urinary low-molecular-weight proteins. Based on these clinical presentations, interstitial nephritis due to Sjögren's syndrome was strongly suspected. Unexpectedly, renal pathology revealed amyloid light-chain (AL) lambda-type depositions predominantly in the vasculatures with severe tubulointerstitial damage. Concentrated urine immunofixation was positive for Bence Jones lambda-type monoclonal proteins...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28176254/-18-fluorine-sodium-fluoride-positron-emission-tomography-a-potential-biomarker-of-transthyretin-cardiac-amyloidosis
#12
Rachelle Morgenstern, Randy Yeh, Adam Castano, Mathew S Maurer, Sabahat Bokhari
BACKGROUND: Non-invasive imaging to diagnose and quantify amyloid load, progression, and response to treatment are central for the care of patients with cardiac amyloidosis. (18)Fluorine-labeled sodium fluoride ((18)F-NaF) is a widely available radioisotope and PET imaging allows for absolute quantification of tracer uptake. METHODS: Patients with biopsy-proven transthyretin (ATTR-CA) and light-chain cardiac amyloidosis (AL) (3 ATTRwt, 2 ATTRV122I, 2 AL) and controls (n = 5), underwent (18)F-NaF PET imaging...
February 7, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28160367/cerebral-amyloidoma-is-characterized-by-b-cell-clonality-and-a-stable-clinical-course
#13
Katharina Heß, Jan Purrucker, Ute Hegenbart, Benjamin Brokinkel, Rouven Berndt, Kathy Keyvani, Camelia M Monoranu, Mario Löhr, Guido Reifenberger, Christopher Munoz-Bendix, Jörg Kalla, Justus Groß, Uta Schick, Jennifer Kollmer, Wolfram Klapper, Christoph Röcken, Martin Hasselblatt, Werner Paulus
Amyloidomas are rare amyloid-containing lesions, which may also occur in the central nervous system. Etiology, pathogenesis and clinical course are poorly understood. To gain more insight into the biology of cerebral amyloidoma, we aimed to characterize its histopathological, molecular and clinical features in a retrospective series of seven patients. FFPE tissue specimens were examined using immunohistochemistry, chromogenic in situ hybridization (CISH) for light chains kappa and lambda as well as an IgH gene clonality analysis...
February 4, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28153807/amyloidosis-a-cancer-derived-paraproteinemia-and-kidney-involvement
#14
REVIEW
Jolanta Małyszko, Klaudia Kozłowska, Jacek Stanisław Małyszko
Amyloidosis is the general term describing the extracellular tissue deposition of fibrils composed of low molecular weight subunits of a variety of proteins. There are multiple different human protein precursors of amyloid fibrils. Amyloid deposits are stained using Congo Red and show typical apple-green birefringence in polarized microscopy. Nowadays, a novel technique LMD/MS technique or laser microdissection combined with mass spectrometry help to diagnose amyloidosis. Amyloidosis of the kidney is typically classified as being either one of two types: AL or AA...
January 30, 2017: Advances in Medical Sciences
https://www.readbyqxmd.com/read/28145535/amyloid-cardiomyopathy
#15
Lucie Karafiatova, Tomas Pika
Amyloidosis is a heterogeneous group of diseases characterized by the deposition of amyloid. It is caused by extracellular deposition of insoluble fibrils with beta-pleated sheet configuration. The protein misfolding abnormalities result in amyloid fibrils and may manifest as primary, secondary, or familial amyloidosis. Amyloid deposition can occur in multiple organs (eg, heart, liver, kidney, skin, eyes, lungs, nervous system) resulting in a variety of clinical manifestations. Cardiac involvement can occur as part of a systemic disease or as a localized phenomenon...
February 1, 2017: Biomedical Papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
https://www.readbyqxmd.com/read/28134587/amyloidosis-of-the-lung
#16
REVIEW
Andras Khoor, Thomas V Colby
CONTEXT: -Amyloidosis is a heterogeneous group of diseases characterized by the deposition of congophilic amyloid fibrils in the extracellular matrix of tissues and organs. To date, 31 fibril proteins have been identified in humans, and it is now recommended that amyloidoses be named after these fibril proteins. Based on this classification scheme, the most common forms of amyloidosis include systemic AL (formerly primary), systemic AA (formerly secondary), systemic wild-type ATTR (formerly age-related or senile systemic), and systemic hereditary ATTR amyloidosis (formerly familial amyloid polyneuropathy)...
February 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28132895/cbp-p300-bromodomain-mediates-amyloid-formation
#17
Daiqing Liao
Bromodomains are protein domains that serve as "readers" of acetylated lysine marks and mediate DNA-templated processes. In this issue of Cell Chemical Biology, Olzscha et al. (2017) report that the CBP/p300 bromodomains mediate the formation of amyloid-like aggregates and that inhibitors specific to these bromodomains reduce the degree of protein aggregation and mitigate HDAC inhibitor-induced cytotoxicity.
January 26, 2017: Cell Chemical Biology
https://www.readbyqxmd.com/read/28132512/cardiac-light-chain-amyloidosis-the-role-of-metal-ions-in-oxidative-stress-and-mitochondrial-damage
#18
Luisa Diomede, Margherita Romeo, Paola Rognoni, Marten Beeg, Claudia Foray, Elena Ghibaudi, Giovanni Palladini, Robert Alan Cherny, Laura Verga, Gian Luca Capello, Vittorio Perfetti, Fabio Fiordaliso, G Merlini, Mario Salmona
<b>Aims: </b>The knowledge of the mechanism underlying the cardiac damage in immunoglobulin light chain (LC) amyloidosis (AL) is essential to develop novel therapies and improve patients' outcome. Although an active role of radical oxygen species (ROS) in LC-induced cardiotoxicity has already been envisaged, the actual mechanisms behind their generation remain elusive. This study was aimed at further dissecting the action of ROS generated by cardiotoxic LC in vivo and investigating whether transition metal ions are involved in this process...
January 28, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/28130138/synthetic-food-additive-dye-tartrazine-triggers-amorphous-aggregation-in-cationic-myoglobin
#19
Nasser Abdulatif Al-Shabib, Javed Masood Khan, Mohd Shahnawaz Khan, Mohd Sajid Ali, Abdulrahman M Al-Senaidy, Mohammad A Alsenaidy, Fohad Mabood Husain, Hamad A Al-Lohedan
Protein aggregation, a characteristic of several neurodegenerative diseases, displays vast conformational diversity from amorphous to amyloid-like aggregates. In this study, we have explored the interaction of tartrazine with myoglobin protein at two different pHs (7.4 and 2.0). We have utilized various spectroscopic techniques (turbidity, Rayleigh light scattering (RLS), intrinsic fluorescence, Congo Red and far-UV CD) along with microscopy techniques i.e. atomic force microscopy (AFM) and transmission electron microscopy (TEM) to characterize the tartrazine-induced aggregation in myoglobin...
January 24, 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/28110969/-acquired-non-amyloid-related-factor%C3%A2-x-deficiency-a-first-case-associated-with-atypical-chronic-lymphocytic-leukemia-and-literature-review
#20
L Boudin, M Patient, E Roméo, J-S Bladé, O Gisserot, J-P de Jauréguiberry
INTRODUCTION: Acquired factor X deficiency is in most cases associated with AL amyloidosis. Acquired non-amyloid related factor X deficiency (DNAA-FX) has been exceptionally reported in the literature. CASE REPORT: We report the first case of acquired, non-amyloid related factor X deficiency associated with atypical chronic lymphoid leukemia in a 66-year-old patient with spontaneous hematomas. After therapeutic failure with polyclonal intravenous immunoglobulins, specific lymphoid malignancy treatment allowed symptoms and coagulation disorder resolution...
January 19, 2017: La Revue de Médecine Interne
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