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systemic amyloidosis

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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
#1
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/29774542/accelerated-pre-senile-systemic-amyloidosis-in-pacap-knockout-mice-a-protective-role-of-pacap-in-age-related-degenerative-processes
#2
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
#3
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
#4
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/29770468/toll-like-receptor-signalling-induces-the-expression-of-serum-amyloid-a-in-epidermal-keratinocytes-and-dermal-fibroblasts
#5
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/29770283/cardiac-amyloidosis-diagnosis-with-magnetic-resonance-imaging-a-case-report
#6
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
#7
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/29762107/musculoskeletal-manifestations-of-amyloidosis-a-focused-review
#8
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/29752392/independent-prognostic-value-of-stroke-volume-index-in-patients-with-immunoglobulin-light-chain-amyloidosis
#9
(no author information available yet)
BACKGROUND: Heart involvement is the most important prognostic determinant in AL amyloidosis patients. Echocardiography is a cornerstone for the diagnosis and provides important prognostic information. METHODS AND RESULTS: We studied 754 patients with AL amyloidosis who underwent echocardiographic assessment at the Mayo Clinic, including a Doppler-derived measurement of stroke volume (SV) within 30 days of their diagnosis to explore the prognostic role of echocardiographic variables in the context of a well-established soluble cardiac biomarker staging system...
May 2018: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29728702/elevated-levels-of-brain-homocysteine-directly-modulate-the-pathological-phenotype-of-a-mouse-model-of-tauopathy
#10
Antonio Di Meco, Jian-Guo Li, Carlos Barrero, Salim Merali, Domenico Praticò
A high circulating level of homocysteine (Hcy), also known as hyperhomocysteinemia, is a risk factor for Alzheimer's disease (AD). Previous studies show that elevated Hcy promotes brain amyloidosis and behavioral deficits in mouse models of AD. However, whether it directly modulates the development of tau neuropathology independently of amyloid beta in vivo is unknown. Herein, we investigate the effect of diet-induced elevated levels of brain Hcy on the phenotype of a relevant mouse model of human tauopathy...
May 4, 2018: Molecular Psychiatry
https://www.readbyqxmd.com/read/29719808/primary-endobronchial-amyloidosis-a-rare-case-of-endobronchial-tumor
#11
Amos Lal, Jamal Akhtar, Mohammad Saud Khan, Yayan Chen, Yaron Goldman
Primary localized endobronchial amyloidosis is a rare entity, as pulmonary amyloidosis most commonly occurs as a part of systemic AL amyloidosis. It can be asymptomatic or can present with nonspecific symptoms such as progressive dyspnea, cough, wheezing and rarely respiratory failure. It is frequently misdiagnosed as asthma, COPD or pneumonia. Solitary endobronchial amyloidosis having a nodular appearance can mimic endobronchial tumor. The diagnosis is usually delayed by 8-37 months. The average life expectancy for primary tracheobronchial amyloidosis is approximately 9 years, so the early diagnosis of this disease is very critical to improve the prognosis of patients...
2018: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/29706127/utility-of-the-18-f-florbetapir-positron-emission-tomography-in-systemic-amyloidosis
#12
Jaume Mestre-Torres, Carles Lorenzo-Bosquet, Gemma Cuberas-Borrós, Mercedes Gironella, Roser Solans-Laque, Andreu Fernández-Codina, Segundo Bujan-Rivas, Joan Castell-Conesa, Fernando Martínez-Valle
Amyloidosis comprises a group of heterogeneous conditions. To ascertain the burden of disease is important because it can determine the treatment as well as the evolution of the disease. Recent reports have shown good results in diagnosing cardiac amyloidosis using 18 F-florbetapir. We hypothesize that combining whole body PET/CT with 18 F-Florbetapir can be useful to characterize the burden of the disease. We included 25 patients, 13 of them with different types of amyloidosis, and 12 with Alzheimer's disease as controls...
April 28, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29701172/tocilizumab-histologically-improved-aa-renal-amyloidosis-in-a-patient-with-multicentric-castleman-disease-a-case-report
#13
Yumi Yamada, Toshinori Ueno, Taisuke Irifuku, Ayumu Nakashima, Shigehiro Doi, Tatsuo Ichinohe, Takao Masaki
Multicentric Castleman disease (MCD) is a rare systemic lymphoproliferative disorder and is infrequently associated with renal complications that include amyloid A (AA) amyloidosis. Although it has been reported that patients with MCD and amyloidosis usually have a poor prognosis, recently, tocilizumab, a humanized anti-interleukin-6 receptor antibody, has emerged as an effective and specific treatment for AA amyloidosis secondary to chronic inflammatory disorders. Here we report a case of an MCD patient with secondary AA renal amyloidosis who was successfully treated with tocilizumab...
April 27, 2018: Clinical Nephrology
https://www.readbyqxmd.com/read/29700868/surface-enhanced-raman-spectroscopy-distinguishes-amyloid-%C3%AE-protein-isoforms-and-conformational-states
#14
Xinke Yu, Eric Y Hayden, Ming Xia, Owen Liang, Lisa Cheah, David B Teplow, Ya-Hong Xie
Amyloid β-protein (Aβ) self-association is one process linked to the development of Alzheimer's disease (AD). Aβ peptides, including its most abundant forms, Aβ40 and Aβ42, are associated with the two predominant neuropathologic findings in AD, vascular and parenchymal amyloidosis, respectively. Efforts to develop therapies for AD often have focused on understanding and controlling the assembly of these two peptides. An obligate step in these efforts is the monitoring of assembly state. We show here that surface-enhanced Raman spectroscopy (SERS) coupled with principal component analysis (PCA) readily distinguishes Aβ40 and Aβ42...
April 26, 2018: Protein Science: a Publication of the Protein Society
https://www.readbyqxmd.com/read/29700757/echocardiographic-and-pathologic-findings-of-wild-type-transthyretin-senile-systemic-amyloidosis-developed-in-early-50s
#15
Goro Yoshioka, Nozomi Watanabe, Hiroyuki Koita, Yujiro Asada, Keiichi Ashikaga, Nehiro Kuriyama, Yoshisato Shibata
No abstract text is available yet for this article.
April 26, 2018: Journal of Echocardiography
https://www.readbyqxmd.com/read/29700090/cardiac-amyloidosis
#16
Ana Martinez-Naharro, Philip N Hawkins, Marianna Fontana
Systemic amyloidosis comprises an uncommon group of disorders caused by the extracellular deposition of misfolded proteins in various organs. Cardiac amyloid deposition, causing an infiltrative/restrictive cardiomyopathy, is a frequent feature of amyloidosis and a major determinant of survival. It may be the presenting feature of the disease or may be identified while investigating a patient presenting with other organ involvement. The need for a high index of suspicion and the critical importance of precise biochemical typing of the amyloid deposits is paramount in light of recent therapeutic advances that can significantly improve prognosis...
April 1, 2018: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/29696194/rationale-and-design-of-dual-study-doxycycline-to-upgrade-response-in-light-chain-al-amyloidosis-dual-a-phase-2-pilot-study-of-a-two-pronged-approach-of-prolonged-doxycycline-with-plasma-cell-directed-therapy-in-the-treatment-of-al-amyloidosis
#17
Anita D'Souza, Kathryn Flynn, Saurabh Chhabra, Binod Dhakal, Mehdi Hamadani, Kirsten Jacobsen, Marcelo Pasquini, Dorothee Weihrauch, Parameswaran Hari
Light chain (AL) amyloidosis is a plasma cell neoplasm associated with insoluble fibril deposition from clonal immunoglobulin chains systemically. The disease is associated with high early mortality and morbidity owing to advanced organ deposition as well as lack of proven de-fibrillogenic therapies. Pre-clinical and retrospective clinical data suggests that doxycycline has benefit in AL amyloidosis. The ongoing DUAL study is a single center, open label, phase 2 study in which patients with AL amyloidosis who are undergoing clone-directed therapy for the underlying neoplasm with oral doxycycline given for 1 year to test the hypothesis that prolonged doxycycline use will be safe, feasible, and lead to reduced early mortality in systemic AL amyloidosis and hasten organ amyloid response...
December 2017: Contemporary Clinical Trials Communications
https://www.readbyqxmd.com/read/29692592/revisiting-renal-amyloidosis-with-clinicopathological-characteristics-grading-and-scoring-a-single-institutional-experience
#18
Abhiram Kalle, Archana Gudipati, Sree Bhushan Raju, Karthik Kalidindi, Swarnalatha Guditi, Gangadhar Taduri, Megha S Uppin
INTRODUCTION: Kidney involvement is a major cause of mortality in systemic amyloidosis. Glomerulus is the most common site of deposition in renal amyloidosis, and nephrotic syndrome is the most common presentation. Distinction between AA and AL is done using immunofluorescence (IF) and immunohistochemistry (IHC). Renal biopsy helps in diagnosis and also predicting the clinical course by applying scoring and grading to the biopsy findings. MATERIALS AND METHODS: The study includes all cases of biopsy-proven renal amyloidosis from January 2008 to May 2017...
April 2018: Journal of Laboratory Physicians
https://www.readbyqxmd.com/read/29683383/lipid-nanoparticles-enabling-gene-therapies-from-concepts-to-clinical-utility
#19
Jayesh A Kulkarni, Pieter R Cullis, Roy van der Meel
Genetic drugs based on RNA or DNA have remarkable therapeutic potential as virtually any disease can be treated by silencing a pathological gene, expressing a beneficial protein, or by editing defective genes. However, therapies based on nucleic acid polymers require sophisticated delivery systems to deliver these macromolecules to the interior of target cells. In this study, we review progress in developing nonviral lipid nanoparticle (LNP) delivery systems that have attractive properties, including ease of manufacture, reduced immune responses, multidosing capabilities, larger payloads, and flexibility of design...
April 23, 2018: Nucleic Acid Therapeutics
https://www.readbyqxmd.com/read/29680336/cardiac-structural-and-functional-consequences-of-amyloid-deposition-by-cardiac-magnetic-resonance-and-echocardiography-and-their-prognostic-roles
#20
Daniel S Knight, Giulia Zumbo, William Barcella, Jennifer A Steeden, Vivek Muthurangu, Ana Martinez-Naharro, Thomas A Treibel, Amna Abdel-Gadir, Heerajnarain Bulluck, Tushar Kotecha, Rohin Francis, Tamer Rezk, Candida C Quarta, Carol J Whelan, Helen J Lachmann, Ashutosh D Wechalekar, Julian D Gillmore, James C Moon, Philip N Hawkins, Marianna Fontana
OBJECTIVES: This cross-sectional study aimed to describe the functional and structural cardiac abnormalities that occur across a spectrum of cardiac amyloidosis burden and to identify the strongest cardiac functional and structural prognostic predictors in amyloidosis using cardiac magnetic resonance (CMR) and echocardiography. BACKGROUND: Cardiac involvement in light chain and transthyretin amyloidosis is the main driver of prognosis and influences treatment strategies...
April 13, 2018: JACC. Cardiovascular Imaging
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