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Amyloidosis

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https://www.readbyqxmd.com/read/29782315/tlr4-gene-expression-and-pro-inflammatory-cytokines-in-alzheimer-s-disease-and-in-response-to-hippocampal-deafferentation-in-rodents
#1
Justin Miron, Cynthia Picard, Josée Frappier, Doris Dea, Louise Théroux, Judes Poirier
One important aspect in Alzheimer's disease pathology is the presence of chronic inflammation. Considering its role as a key receptor in the microglial innate immune system, TLR4 was shown to regulate the binding and phagocytosis of amyloid plaques by microglia in several mouse models of amyloidosis, as well as the production of pro-inflammatory cytokines. To our knowledge, TLR4 and its association with cytokines have not been thoroughly examined in the brains of subjects affected with Alzheimer's disease. Using quantitative reverse transcription polymerase chain reaction (qRT-PCR) in postmortem human brains, we observed increased expression for the TLR4 and TNF genes (p = 0...
May 16, 2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29782249/-transthyretin-amyloidosis-in-a-cohort-of-old-and-very-old-patients-with-chronic-heart-failure
#2
A A Poliakova, E N Semernin, M Y Sitnikova, K L Аvagyan, R V Grozov, S A Pyko, A N Krutikov, V G Davydova, K A Khmelnitskaya, M M Shavloskii, D E Korzhevskii, A Y Gudkova
BACKGROUND: Life-time diagnostics of wild type transthyretin amyloidosis (ATTR(wt)-amyloidosis) is virtually absent, even though ATTR(wt)-amyloidosis is an underestimated cause for morbidity and mortality, particularly in the older age group. AIM: To study incidence, demographic characteristics, and morpho-functional features of ATTR(wt)-amyloidosis in patients with FC IV CHF and LV hypertrophy > 15 mm according to autopsy data. MATERIALS AND METHODS: Postmortem reports were retrospectively analyzed for patients (n=141; 19 % males, 81 % females) of cardiology departments aged ≥69 with the underlying CHF syndrome...
2018: Kardiologiia
https://www.readbyqxmd.com/read/29780861/the-relationship-between-recall-of-recently-versus-remotely-encoded-famous-faces-and-amyloidosis-in-clinically-normal-older-adults
#3
Irina Orlovsky, Willem Huijbers, Bernard J Hanseeuw, Elizabeth C Mormino, Trey Hedden, Rachel F Buckley, Molly LaPoint, Jennifer S Rabin, Dorene M Rentz, Keith A Johnson, Reisa A Sperling, Kathryn V Papp
Introduction: Alzheimer's disease (AD) patients exhibit temporally graded memory loss with remote memories remaining more intact than recent memories. It is unclear whether this temporal pattern is observable in clinically normal adults with amyloid pathology (i.e. preclinical AD). Methods: Participants were asked to recall the names of famous figures most prominent recently (famous after 1990) and remotely (famous from 1960-1980) and were provided with a phonemic cue to ensure that memory failure was not purely due to verbal retrieval weaknesses...
2018: Alzheimer's & Dementia: Diagnosis, Assessment & Disease Monitoring
https://www.readbyqxmd.com/read/29779881/failure-of-tafamidis-to-halt-progression-of-ala36pro-ttr-oculomeningovascular-amyloidosis
#4
Fabrizio Salvi, Roberto Volpe, Francesca Pastorelli, Andrea Bianchi, Alessandra Vella, Claudio Rapezzi, Mario Mascalchi
Oculomeningovascular amyloidosis is a variant of transthyretin (TTR) amyloidotic polyneuropathy, which is associated with blindness and brain ischemia, microhemorrages, and siderosis due to prominent production of the abnormal TTR in the eye and in the choroid plexuses. Tafamidis is a TTR stabilizer that is orally administered and, by interfering with amyloid fibril formation and deposition, is capable of slowing progression of TTR polyneuropathy and of early-stage cardiomyopathy. However, the ocular manifestations of amyloid deposition progressed despite tafamidis therapy in Val30Met TTR amyloidosis, and the effects of tafamidis on meningovascular amyloidosis are unknown...
May 17, 2018: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/29777721/pentraxins-crp-i-and-crp-ii-are-post-translationally-deiminated-and-differ-in-tissue-specificity-in-cod-gadus-morhua-l-ontogeny
#5
Bergljót Magnadóttir, Polly Hayes, Berglind Gísladóttir, Birkir Þór Bragason, Mariya Hristova, Anthony P Nicholas, Sigríður Guðmundsdóttir, Sigrun Lange
Pentraxins are fluid phase pattern recognition molecules that form an important part of the innate immune defence and are conserved between fish and human. In Atlantic cod (Gadus morhua L.), two pentraxin-like proteins have been described, CRP-I and CRP-II. Here we show for the first time that these two CRP forms are post-translationally deiminated (an irreversible conversion of arginine to citrulline) and differ with respect to tissue specific localisation in cod ontogeny from 3 to 84 days post hatching. While both forms are expressed in liver, albeit at temporally differing levels, CRP-I shows a strong association with nervous tissue while CRP-II is strongly associated to mucosal tissues of gut and skin...
May 16, 2018: Developmental and Comparative Immunology
https://www.readbyqxmd.com/read/29777480/the-evolving-landscape-of-nuclear-imaging-in-cardiac-amyloidosis
#6
EDITORIAL
Steven J Promislow, Terrence D Ruddy
No abstract text is available yet for this article.
May 18, 2018: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/29775082/profile-of-renal-aa-amyloidosis-in-older-and-younger-individuals-a-single-centre-experience
#7
Siyar Erdogmus, Zeynep Kendi Celebi, Serkan Akturk, Gizem Kumru, Neval Duman, Kenan Ates, Sehsuvar Erturk, Gokhan Nergizoglu, Sim Kutlay, Sule Sengul, Kenan Keven
OBJECTIVE: In epidemiological studies of amyloid A (AA) amyloidosis from Turkey, the most frequently cause was familial Mediterranean fever (FMF) and it occurs generally in young age population. However, there are no sufficient data regarding aetiology, clinical presentation and prognosis of renal AA amyloidosis in advanced age patients. In this study, we aimed to investigate demographic, clinical presentation, aetiology and outcomes of adults aged 60 years or older patients with biopsy-proven renal AA amyloidosis...
May 18, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29774542/accelerated-pre-senile-systemic-amyloidosis-in-pacap-knockout-mice-a-protective-role-of-pacap-in-age-related-degenerative-processes
#8
Dora Reglodi, Adel Jungling, Rémi Longuespée, Joerg Kriegsmann, Rita Casadonte, Mark Kriegsmann, Tamas Juhasz, Sebastian Bardosi, Andrea Tamas, Balazs Daniel Fulop, Krisztina Kovacs, Zsuzsanna Nagy, Jason Sparks, Attila Miseta, Gabriel Mazzucchelli, Hitoshi Hashimoto, Attila Bardosi
Dysregulation of neuropeptides may play an important role in aging-induced impairments. Among them, pituitary adenylate cyclase activating polypeptide (PACAP) is a potent cytoprotective peptide that provides an endogenous control against a variety of tissue-damaging stimuli. We hypothesized that the progressive decline of PACAP throughout life, and the well-known general cytoprotective effects of PACAP lead to age-related pathophysiological changes in PACAP deficiency, supported by the increased vulnerability to various stressors of animals partially or totally lacking PACAP...
May 17, 2018: Journal of Pathology
https://www.readbyqxmd.com/read/29773275/periodic-fever-syndromes
#9
REVIEW
Helen J Lachmann
Periodic fever syndromes are autoinflammatory diseases. The majority present in infancy or childhood and are characterised by recurrent episodes of fever and systemic inflammation that occur in the absence of autoantibody production or identifiable infection. The best recognised disorders include CAPS, FMF, TRAPS and MKD. Understanding the molecular pathogenesis of these disorders provides unique insights into the regulation of innate immunity. Diagnosis relies on clinical acumen and is supported by genetic testing...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29772028/randomized-phase-i-trial-hiv-core-003-depletion-of-serum-amyloid-p-component-and-immunogenicity-of-dna-vaccination-against-hiv-1
#10
Nicola J Borthwick, Thirusha Lane, Nathifa Moyo, Alison Crook, Jung Min Shim, Ian Baines, Edmund G Wee, Philip N Hawkins, Julian D Gillmore, Tomáš Hanke, Mark B Pepys
BACKGROUND: The failure of DNA vaccination in humans, in contrast to its efficacy in some species, is unexplained. Observational and interventional experimental evidence suggests that DNA immunogenicity may be prevented by binding of human serum amyloid P component (SAP). SAP is the single normal DNA binding protein in human plasma. The drug (R)-1-[6-[(R)-2-carboxypyrrolidin-1-yl]-6-oxo-hexanoyl]pyrrolidine-2-carboxylic acid (CPHPC, miridesap), developed for treatment of systemic amyloidosis and Alzheimer's disease, depletes circulating SAP by 95-99%...
2018: PloS One
https://www.readbyqxmd.com/read/29770800/current-and-future-circulating-biomarkers-for-cardiac-amyloidosis
#11
REVIEW
Marco Luciani, Luca Troncone, Federica Del Monte
Cardiac amyloidosis (CA) comprises a heterogeneous group of medical conditions affecting the myocardium. It presents with proteinaceous infiltration with variable degrees of severity, prevalence and evolution. Despite this heterogeneity, erroneous protein folding is the common pathophysiologic process, yielding the formation of a single misfolded protein (monomer) that progressively evolves and ultimately forms amyloid fibers. Additionally, by seeding out from the organs of origin, intermediates called oligomers metastasize and restart the process...
May 17, 2018: Acta Pharmacologica Sinica
https://www.readbyqxmd.com/read/29770468/toll-like-receptor-signalling-induces-the-expression-of-serum-amyloid-a-in-epidermal-keratinocytes-and-dermal-fibroblasts
#12
S Morizane, A Kajita, K Mizuno, T Takiguchi, K Iwatsuki
BACKGROUND: Toll-like receptors (TLRs) play critical roles in innate immune response by sensing pathogen- or damage-associated molecular patterns. Epidermal keratinocytes and dermal fibroblasts also produce proinflammatory cytokines and chemokines under stimulation with TLR ligands. Serum amyloid A (SAA) is an essential factor in the pathogenesis of secondary amyloidosis, and also has immunomodulatory functions. SAA are produced mainly by hepatocytes but also by a variety of cells, including immune cells, endothelial cells, synoviocytes, and epidermal keratinocytes...
May 16, 2018: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/29770336/epigallocatechin-3-gallate-reduces-neuronal-apoptosis-in-rats-after-middle-cerebral-artery-occlusion-injury-via-pi3k-akt-enos-signaling-pathway
#13
Wang Nan, Xu Zhonghang, Chen Keyan, Liu Tongtong, Guo Wanshu, Xu Zhongxin
Background/Aims: Epigallocatechin-3-gallate (EGCG) has neuroprotective effects and the ability to resist amyloidosis. This study observed the protective effect of EGCG against neuronal injury in rat models of middle cerebral artery occlusion (MCAO) and investigated the mechanism of action of PI3K/AKT/eNOS signaling pathway. Methods: Rat models of permanent MCAO were established using the suture method. Rat behavior was measured using neurological deficit score. Pathology and apoptosis were measured using HE staining and TUNEL...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29770283/cardiac-amyloidosis-diagnosis-with-magnetic-resonance-imaging-a-case-report
#14
Meidi El Issa, Malik El Issa, Besma Sidia
Amyloidosis is a rare disease, which can affect various organs, such as the kidneys, heart, liver, respiratory and gastrointestinal tracts, and the nervous system. It still has a bad prognosis nowadays, despite chemotherapy and the new biotherapies. Its physiopathology corresponds to an irreversible, extracellular accumulation of fibrillous proteins in the tissues. Notwithstanding the fact that a clear diagnosis can be made with histology (of solid injured organs or a subcutaneous biopsy), magnetic resonance imaging (MRI) can show various advantages, especially to prove cardiac involvement, with great specificity and sensibility as well...
March 15, 2018: Curēus
https://www.readbyqxmd.com/read/29767440/successful-treatment-of-systemic-aa-amyloidosis-associated-with-underlying-hodgkin-lymphoma
#15
Richa Manwani, David Wrench, Ashutosh Wechalekar, Helen Lachmann
No abstract text is available yet for this article.
May 16, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29767409/the-use-of-serum-free-light-chain-dimerization-patterns-assist-in-the-diagnosis-of-al-amyloidosis
#16
Moshe E Gatt, Batia Kaplan, Dean Yogev, Elana Slyusarevsky, Galina Pogrebijski, Sizilia Golderman, Olga Kukuy, Avi Livneh
The discrimination between benign and malignant forms of plasma cell dyscrasia (PCD) is often difficult. Free light chain monomer-dimer pattern analysis (FLC-MDPA) may assist in solving this dilemma and distinguish between AL amyloidosis and benign PCD. Serum samples of patients with AL amyloidosis and benign PCD were analysed in a blinded manner. Quantitative Western blotting was performed to estimate dimerization and clonality indices, and thereby determine the source of the tested samples, as derived either from benign or malignant PCD...
May 16, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29762107/musculoskeletal-manifestations-of-amyloidosis-a-focused-review
#17
Thomas X Nguyen, Abbas Naqvi, Terry L Thompson, Robert H Wilson
Amyloidosis is a poorly understood condition that can wreak havoc on numerous systems within the human body. In addition, this disease can present in multiple forms which each have their own unique physiology and subsequent effects. However, while the literature on the etiology and effect of amyloidosis on various organ systems is numerous, few have highlighted the musculoskeletal manifestations of this devastating disease. This review focuses on the recent research on amyloid deposition in the musculoskeletal system...
2018: Journal of Surgical Orthopaedic Advances
https://www.readbyqxmd.com/read/29762014/selective-and-sensitive-pull-down-of-amyloid-fibrils-produced-in-vitro-and-in-vivo-by-the-use-of-pentameric-thiophene-coupled-resins
#18
Anna Beatriz Wreden, Luiza Fernandes, Mirian Kelley, Antônio Pereira-Neves, Caroline S Moreira, David R da Rocha, Fernando L Palhano
Protein aggregation is a hallmark of several degenerative diseases, including Alzheimer's disease, Parkinson's disease and familial amyloidosis (Finnish type) (FAF). A method to isolate and detect amyloids is desired for the diagnosis of amyloid diseases. Here, we report the synthesis of pentameric thiophene amyloid ligand (p-FTAA) linked to agarose resin for selective purification of amyloid aggregates produced in vitro and in vivo. Using amyloid fibrils produced in vitro from alpha-synuclein, gelsolin and Aβ1-40 and gelsolin amyloid aggregates extracted from tissue homogenates of a mouse model of FAF, we observed that p-FTAA resin was able to pull down amyloid aggregates...
May 15, 2018: ACS Chemical Neuroscience
https://www.readbyqxmd.com/read/29758211/localized-tracheal-amyloidosis
#19
Luis Gorospe, Rosa María Gómez-García, Paola Arrieta, Alberto Cabañero-Sánchez, Odile Ajuria-Illarramendi, Esther Gambí-Pisonero
No abstract text is available yet for this article.
May 11, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29756110/agenesis-of-the-isthmus-of-the-thyroid-gland
#20
Uğur Kesici, Sevgi Kesici
The thyroid is an endocrine gland composed of two lobes connected by the isthmus tissue. Thyroid isthmus agenesis is a rare condition, and only a few cases have been reported in the literature. Here, we discuss the case of a 56-year-old female patient in whom agenesis of the thyroid isthmus was discovered incidentally during surgery for a multinodular goitre. When agenesis of the isthmus is found, associated thyroid lobe agenesis and the presence of ectopic thyroid tissue must be considered. In addition, associated autoimmune thyroid nodule, thyroiditis, primary thyroid carcinoma, metastasis, and amyloidosis must be considered in the differential diagnosis...
2018: Turkish Journal of Surgery
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