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Al amyloid

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https://www.readbyqxmd.com/read/27900617/new-and-evolving-concepts-regarding-the-prognosis-and-treatment-of-cardiac-amyloidosis
#1
REVIEW
Stefano Perlini, Roberta Mussinelli, Francesco Salinaro
Systemic amyloidoses are rare and proteiform diseases, caused by extracellular accumulation of insoluble misfolded fibrillar proteins. Prognosis is dictated by cardiac involvement, which is especially frequent in light chain (AL) and in transthyretin variants (ATTR, both mutated, (ATTRm), and wild-type, (ATTRwt)). Recently, ATTRwt has emerged as a potentially relevant cause of a heart failure with preserved ejection fraction (HFpEF). Cardiac amyloidosis is an archetypal example of restrictive cardiomyopathy, with signs and symptoms of global heart failure and diastolic dysfunction...
November 29, 2016: Current Heart Failure Reports
https://www.readbyqxmd.com/read/27893738/fenugreek-seed-powder-nullified-aluminium-chloride-induced-memory-loss-biochemical-changes-a%C3%AE-burden-and-apoptosis-via-regulating-akt-gsk3%C3%AE-signaling-pathway
#2
Asokan Prema, Arokiasamy Justin Thenmozhi, Thamilarasan Manivasagam, Musthafa Mohamed Essa, Mohammed D Akbar, Mohammed Akbar
Alzheimer's disease (AD) is the most common form of dementia that mainly affects the cognitive functions of the aged populations. Trigonella foenum-graecum (L.) (fenugreek), a traditionally well utilized medicinal plant ubiquitously used as one of the main food additive worldwide, is known to have numerous beneficial health effects. Fenugreek seed extract could be able to inhibit the activity of acetylcholinesterase (AChE), a key enzyme involved in the pathogenesis of AD, and further shown to have anti-parkinsonic effect...
2016: PloS One
https://www.readbyqxmd.com/read/27884212/angiotensin-converting-enzyme-2-is-reduced-in-alzheimer-s-disease-in-association-with-increasing-amyloid-%C3%AE-and-tau-pathology
#3
Patrick Gavin Kehoe, Steffenny Wong, Noura Al Mulhim, Laura Elyse Palmer, J Scott Miners
BACKGROUND: Hyperactivity of the classical axis of the renin-angiotensin system (RAS), mediated by angiotensin II (Ang II) activation of the angiotensin II type 1 receptor (AT1R), is implicated in the pathogenesis of Alzheimer's disease (AD). Angiotensin-converting enzyme-2 (ACE-2) degrades Ang II to angiotensin 1-7 (Ang (1-7)) and counter-regulates the classical axis of RAS. We have investigated the expression and distribution of ACE-2 in post-mortem human brain tissue in relation to AD pathology and classical RAS axis activity...
November 25, 2016: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/27872470/hereditary-amyloidosis-with-recurrent-lung-infiltrates
#4
Alberto E Revelo, Crischelle Magaspi, George Maguire, Wilbert S Aronow
BACKGROUND Amyloidosis is a protein conformational disorder characterized by extracellular deposition of amyloid fibrils in extracellular tissue. Lung involvement is most commonly caused by secondary AL amyloidosis. The familial autosomal-dominant senile transthyretin (ATTR) disease manifests mainly as polyneuropathy and restrictive cardiomyopathy denoting the name familial amyloidotic polyneuropathy (FAP). Rarely, this form manifests with clinical and radiologically relevant respiratory tract symptoms and lung involvement...
November 22, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27861403/localized-immunoglobulin-light-chain-amyloid-of-the-conjunctiva-confirmed-by-mass-spectrometry-without-evidence-of-systemic-disease
#5
Eric B Hamill, Preeti J Thyparampil, Michael T Yen
Current guidelines recommend a thorough systemic workup in patients with newly diagnosed immunoglobulin amyloid light chain (AL) amyloidosis because of a close association with an underlying plasma cell proliferative disorder. Rarely, however, AL amyloidosis may present as a localized, benign disease without systemic involvement. Few previous reports have documented localized AL amyloid of the conjunctiva, and all these used immunohistochemistry for amyloid typing, which can be inaccurate. The authors identified 2 cases of AL amyloidosis confirmed by mass spectrometry localized to the conjunctiva and without evidence of systemic disease as determined by thorough systemic workup...
November 15, 2016: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/27847962/the-multi-target-effects-of-cni-1493-convergence-of-anti-amylodogenic-and-anti-inflammatory-properties-in-animal-models-of-alzheimer-s-disease
#6
Roman Sankowski, Arne Herring, Kathy Keyvani, Kathrin Frenzel, Jinyu Wu, Stephan Röskam, Carmen Noelker, Michael Bacher, Yousef Al-Abed
After several decades of Alzheimer's disease (AD) research and failed clinical trials, one can speculate that targeting a single pathway is not sufficient. However, a cocktail of novel therapeutics will constitute a challenging clinical trial. A more plausible approach will capitalize on a drug that has relevant and synergistic multiple-target effects in AD. We have previously demonstrated the efficacy of CNI-1493 in the CRND8 transgenic AD mouse model. Similar to many anti-inflammatory drugs that were tested in preclinical model of AD, it was speculated that the significant effect of CNI-1493 is due to its established anti-inflammatory properties in rodents and humans...
November 15, 2016: Molecular Medicine
https://www.readbyqxmd.com/read/27825470/monoclonal-igm-related-al-amyloidosis
#7
REVIEW
Paolo Milani, Giampaolo Merlini
Monoclonal immunoglobulin M (IgM)-related light chain (AL) amyloidosis, which accounts for 5%-7% of all AL amyloidosis cases, is a distinct clinical entity that poses specific challenges to clinicians. Several studies reported that although there is a substantial overlap, the pattern of organ involvement is peculiar, with higher frequencies of lung, lymph nodes, and peripheral nervous system involvement. A recent collaborative study from three European referral centers, defined that cardiac involvement, advanced Mayo disease stage, neuropathic, and liver involvement were independent factors that had impact on survival in IgM-AL amyloidosis patients...
June 2016: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/27810931/immunotherapy-with-aducanumab-restores-calcium-homeostasis-in-tg2576-mice
#8
Ksenia V Kastanenka, Thierry Bussiere, Naomi Shakerdge, Fang Qian, Paul H Weinreb, Ken Rhodes, Brian J Bacskai
: Calcium homeostasis plays a major role in maintaining neuronal function under physiological conditions. Amyloid-β (Aβ) initiates pathological processes that include disruption in intracellular calcium levels, and therefore amelioration of the calcium alteration could serve as an indirect functional indicator of treatment efficacy. Thus, calcium dynamics was used as a measure of functional outcome. We evaluated the effects of the anti-Aβ antibody aducanumab, on calcium homeostasis and plaque clearance in aged Tg2576 mice with in vivo multiphoton imaging...
November 3, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/27809600/plasma-hepatocyte-growth-factor-is-a-novel-marker-of-al-cardiac-amyloidosis
#9
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/27802241/modulation-of-parkinson-s-disease-associated-protein-rescues-alzheimer-s-disease-degeneration
#10
Akihiko Nunomura, Xiongwei Zhu, George Perry
DJ-1, a causative gene product of an autosomal recessive familial form of Parkinson's disease (PD), plays roles in reducing oxidative stress and transcriptional regulation. Loss of its function is thought to result in the onset of PD. DJ-1 has been demonstrated to show general cytoprotective function mainly through antioxidant properties and possibly regulates the extent of stroke-induced damage and neurodegeneration in Alzheimer's disease (AD). The paper, "Effects of a DJ-1-Binding Compound on Spatial Learning and Memory Impairment in a Mouse Model of Alzheimer's Disease", by Kitamura et al...
October 12, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27790444/amyloidosis-the-newer-discovered-alect2-associated-with-der7q-add-7
#11
Amrita Chakrabarti, Priyanka Samal, Joydeep Chakrabartty
Amyloidosis is characterized by pathological deposition of abnormal protein aggregates in various tissues, AL protein being the commonest. ALECT 2 is the newest protein described, having a predisposition to affect the kidneys, sometimes the liver and rarely other organs. We present a case of renal amyloid ALECT 2 due to leucocyte cell derived chemotaxin 2, a novel amyloidogenic protein. The patient presented with mild proteinuria, scattered plasma cells on bone marrow examination and altered kappa/lambda ratio with associated cytogenetic abnormality of der7q add(7)...
September 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27780035/rna-seeded-functional-amyloids-balance-growth-and-survival
#12
Shawn M Lyons, Paul Anderson
In this issue of Developmental Cell, Audas et al. (2016) report non-membrane-enclosed amyloid bodies (ABs) assembled in the nuclei of cells exposed to heat and low pH. Remarkably, ABs form not by liquid-to-liquid phase separation, implicated in RNA-seeded granule assembly, but by an amyloidogenic process that promotes a liquid-to-solid transition.
October 24, 2016: Developmental Cell
https://www.readbyqxmd.com/read/27766286/dataset-of-the-molecular-dynamics-simulations-of-bilayers-consisting-of-short-amyloidogenic-peptide-vdswnvlvag-from-bgl2p-glucantransferase-of-s-cerevisiae-cell-wall
#13
Anna V Glyakina, Nikolai K Balabaev, Oxana V Galzitskaya
The amyloidogenic peptide VDSWNVLVAG from Bgl2p-glucantransferase of Saccharomyces cerevisiae cell wall and its modifying analog VESWNVLVAG were taken for the construction of four types of bilayers which differ by orientation of the peptides in the layers and of the layers relative to each other. These bilayers were used as starting models for the molecular dynamics (MD) at three charge states (neutral, pH3, and pH5). The changes of the fraction of secondary structure during 1 ns simulations were received for 96 MD trajectories...
December 2016: Data in Brief
https://www.readbyqxmd.com/read/27764008/primary-laryngotracheal-amyloidosis-with-bilateral-vocal-cord-involvement-and-associated-bronchiectasis
#14
Charles W Lanks, Timothy L Van Natta, David W Hsia
Localized tracheobronchial amyloidosis is a rare disease that results from submucosal deposition of insoluble amyloid proteins in the large airways. Amyloidosis affecting the larynx and subglottic space typically results in unilateral, nodular vocal cord infiltration. It rarely can present with bilateral vocal cord involvement and can progress to lifethreatening respiratory failure due to upper airway obstruction. In these patients, typical treatment modalities such as CO2 laser ablation are often ineffectual...
October 2016: Journal of Bronchology & Interventional Pulmonology
https://www.readbyqxmd.com/read/27760336/amyloid-cascade-into-clarity
#15
Tara M Weitz, Terrence Town
In a recent issue of Nature, Sevigny et al. (2016) report findings from a phase 1b clinical trial of aducanumab (a monoclonal antibody targeting misfolded amyloid-β peptides), revitalizing the "amyloid cascade hypothesis" and bringing mononuclear phagocytes center stage in the treatment of Alzheimer's disease.
October 18, 2016: Immunity
https://www.readbyqxmd.com/read/27752386/a-case-of-abdominal-aortic-retroperitoneal-and-mesenteric-amyloid-light-chain-amyloidoma
#16
Kazuhiro Yokota, Dai Kishida, Hidekazu Kayano, Masahide Yazaki, Yuki Shimada, Yuji Akiyama, Toshihide Mimura
We report the case of a Japanese woman with amyloid light chain (AL) amyloidoma in the abdominal aortic retroperitoneum and mesentery. Irregular soft tissue mass lesions with calcification in the abdominal aortic retroperitoneum and mesentery were initially detected by computed tomography at another hospital. The lesions gradually compressed the duodenum, causing symptoms of bowel obstruction. The patient was clinically diagnosed with retroperitoneal fibrosis, and prednisolone was administered at a dose of 40 mg/day...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27744229/effects-of-oxidative-post-translational-modifications-on-structural-stability-and-self-assembly-of-%C3%AE-6-immunoglobulin-light-chain
#17
Ximena Zottig, Mathieu Laporte Wolwertz, Makan Golizeh, Leanne Ohlund, Lekha Sleno, Steve Bourgault
Light chain amyloidosis (AL) originates from the deposition of immunoglobulin light chains (LCs) as amyloid fibrils in the extracellular space of vital organs. Although non-enzymatic post-translational modifications (PTMs) have been shown to contribute to protein misfolding diseases, little is known about their contributions to LC amyloidogenicity. In this study, we investigated the effects of three oxidative PTMs, carbonylation by hydroxynonenal (HNE), oxidation and nitration, on the structure, thermodynamic stability and self-assembly propensity of a LC variable domain from the λ6 germline, Wil...
October 11, 2016: Biophysical Chemistry
https://www.readbyqxmd.com/read/27742814/progression-of-brain-atrophy-in-psp-and-cbs-over-6-months-and-1-year
#18
Shubir Dutt, Richard J Binney, Hilary W Heuer, Phi Luong, Suneth Attygalle, Priyanka Bhatt, Gabe A Marx, Jonathan Elofson, Maria C Tartaglia, Irene Litvan, Scott M McGinnis, Bradford C Dickerson, John Kornak, Dana Waltzman, Lisa Voltarelli, Norbert Schuff, Gil D Rabinovici, Joel H Kramer, Clifford R Jack, Bruce L Miller, Howard J Rosen, Adam L Boxer
OBJECTIVE: To examine the utility and reliability of volumetric MRI in measuring disease progression in the 4 repeat tauopathies, progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), to support clinical development of new tau-directed therapeutic agents. METHODS: Six- and 12-month changes in regional MRI volumes and PSP Rating Scale scores were examined in 55 patients with PSP and 33 patients with CBS (78% amyloid PET negative) compared to 30 normal controls from a multicenter natural history study...
November 8, 2016: Neurology
https://www.readbyqxmd.com/read/27696268/immunoglobulin-light-chain-systemic-amyloidosis
#19
Angela Dispenzieri, Giampaolo Merlini
Immunoglobulin light chain amyloidosis (AL) is a rare, complex disease caused by misfolded free light chains produced by a usually small, indolent plasma cell clone. Effective treatments exist that can alter the natural history, provided that they are started before irreversible organ damage has occurred. The cornerstones of the management of AL amyloidosis are early diagnosis, accurate typing, appropriate risk-adapted therapy, tight follow-up, and effective supportive treatment. The suppression of the amyloidogenic light chains using the cardiac biomarkers as guide to choose chemotherapy is still the mainstay of therapy...
2016: Cancer Treatment and Research
https://www.readbyqxmd.com/read/27676646/evidence-of-both-von-willebrand-factor-deposition-and-factor-v-deposition-onto-al-amyloid-as-the-cause-of-a-severe-bleeding-diathesis
#20
Jonathan S Harrison, Shellaine R Frazier, Dianne D McConnell, Senan John Yasar, Nataliya Melnyk, Gratian Salaru
Acquired coagulopathies are common; uncommonly, adsorption of coagulation factors from the circulation into the tissues by pathologic amyloid exceeds the body's ability to produce factor and results in acquired factor deficiency. When amyloidosis does cause a coagulopathy, it is most often acquired factor X deficiency, but there are rare reports of amyloidosis being associated with other acquired factor deficiencies. We investigated a case of a severe bleeding diathesis, the cause of which was combined acquired factor V deficiency and concomitant acquired von Willebrand syndrome...
September 24, 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
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