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https://www.readbyqxmd.com/read/28528458/cardiac-amyloidosis-diagnosis-and-treatment-strategies
#1
REVIEW
Mirela Tuzovic, Eric H Yang, Arnold S Baas, Eugene C Depasquale, Mario C Deng, Daniel Cruz, Gabriel Vorobiof
Cardiac amyloidosis in the United States is most often due to myocardial infiltration by immunoglobulin protein, such as in AL amyloidosis, or by the protein transthyretin, such as in hereditary and senile amyloidosis. Cardiac amyloidosis often portends a poor prognosis especially in patients with systemic AL amyloidosis. Despite better understanding of the pathophysiology of amyloid, many patients are still diagnosed late in the disease course. This review investigates the current understanding and new research on the diagnosis and treatment strategies in patients with cardiac amyloidosis...
July 2017: Current Oncology Reports
https://www.readbyqxmd.com/read/28527045/amyotrophic-lateral-sclerosis-like-superoxide-dismutase-1-proteinopathy-is-associated-with-neuronal-loss-in-parkinson-s-disease-brain
#2
Benjamin G Trist, Katherine M Davies, Veronica Cottam, Sian Genoud, Richard Ortega, Stéphane Roudeau, Asuncion Carmona, Kasun De Silva, Valerie Wasinger, Simon J G Lewis, Perminder Sachdev, Bradley Smith, Claire Troakes, Caroline Vance, Christopher Shaw, Safa Al-Sarraj, Helen J Ball, Glenda M Halliday, Dominic J Hare, Kay L Double
Neuronal loss in numerous neurodegenerative disorders has been linked to protein aggregation and oxidative stress. Emerging data regarding overlapping proteinopathy in traditionally distinct neurodegenerative diseases suggest that disease-modifying treatments targeting these pathological features may exhibit efficacy across multiple disorders. Here, we describe proteinopathy distinct from classic synucleinopathy, predominantly comprised of the anti-oxidant enzyme superoxide dismutase-1 (SOD1), in the Parkinson's disease brain...
May 19, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28505007/al-amyloidoma-of-the-skin-subcutis-cutaneous-amyloidosis-plasma-cell-dyscrasia-or-a-manifestation-of-primary-cutaneous-marginal-zone-lymphoma
#3
Noreen M Walsh, Ian Marie Lano, Peter Green, Christopher Gallant, Sylvia Pasternak, Thai Yen Ly, Luis Requena, Heinz Kutzner, Andreas Chott, Lorenzo Cerroni
It is unclear whether AL amyloidoma of the skin/subcutis represents a distinct entity, an indolent precursor of systemic amyloidosis, or a manifestation of cutaneous marginal zone lymphoma (cMZL). We collected 10 cases of cutaneous AL amyloidoma in order to better characterize the clinicopathologic features of this elusive entity (M:F=4:6; median age: 62.5 y, range: 31 to 82 y). Nine patients had a solitary nodule or plaque on the lower extremity (n=7), upper extremity (n=1), or chin (n=1). One patient had an AL amyloidoma on the right thigh and a second lesion on the right arm showing histopathologic features of cMZL without amyloid deposits...
May 12, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28490248/sodium-louroyl-sarcosinate-sarkosyl-modulate-amyloid-fibril-formation-in-hen-egg-white-lysozyme-hewl-at-alkaline-ph-a-molecular-insight-study
#4
Javed Masood Khan, Mohd Shahnawaz Khan, Mohammad Abdurehman Alsenaidy, Anwar Ahmed, Priyankar Sen, Mohammad Oves, Nasser Abdulatif Al-Shabib, Rizwan Hasan Khan
Amyloid fibril formation is responsible for several neurodegenerative diseases and are formed when native proteins misfold and stick together with different interactive forces. In the present study, we have determined the mode of interaction of the anionic surfactant sarkosyl with hen egg white lysozyme (HEWL) [EC no. 3.2.1.17] at two pHs (9.0 and 13.0) and investigated its impact on fibrillogenesis. Our data suggested that sarkosyl is promoting amyloid fibril formation in HEWL at the concentration range between 0...
May 10, 2017: Journal of Biomolecular Structure & Dynamics
https://www.readbyqxmd.com/read/28483059/presentation-and-outcomes-of-localized-immunoglobulin-light-chain-amyloidosis-the-mayo-clinic-experience
#5
Taxiarchis V Kourelis, Robert A Kyle, David Dingli, Francis K Buadi, Shaji K Kumar, Morie A Gertz, Martha Q Lacy, Prashant Kapoor, Ronald S Go, Wilson I Gonsalves, Rahma Warsame, John A Lust, Suzanne R Hayman, S Vincent Rajkumar, Steven R Zeldenrust, Stephen J Russell, Yi Lin, Nelson Leung, Angela Dispenzieri
OBJECTIVE: To describe treatment types, outcomes, and relapse patterns in patients with localized immunoglobulin light chain amyloidosis (ALL). PATIENTS AND METHODS: We included all patients with ALL seen at Mayo Clinic in Rochester, Minnesota, from January 1, 1968, through June 30, 2014. The diagnosis of ALL was predicated on the presence of a Congo red-positive biopsy specimen and negative serum and urine immunofixation. Treatment response categories were response, stability, and progression...
May 5, 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28467313/igg-kappa-monoclonal-gammopathy-of-unknown-significance-with-al-amyloidosis-simulating-giant-cell-arteritis
#6
V M Pompilian, S Tanaseanu, Camelia Badea, Sabina Zurac, C Socoliuc, Sorina Badelita, Elena Botez, Mihaela Antohe
Monoclonal gammopathies complicated by AL amyloidosis can mimic giant cell arteritis (GCA).We hereby present the case of a 63 year old woman in whom symptoms consistent with GCA were the first manifestations of a monoclonal gammopathy of unknown significance (MGUS) associated with amyloidosis. A 63 year old woman was admitted for temporal headache, maseterine claudication, neck and shoulder stiffness. She was recently diagnosed with carpal tunnel syndrome. On physical examination she had prominent temporal arteries, macroglosia and orthostatic hypotension...
May 3, 2017: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
https://www.readbyqxmd.com/read/28466190/microglia-endocytose-amyloid-%C3%AE-through-the-binding-of-transglutaminase-2-and-milk-fat-globule-egf-factor-8-protein
#7
Kenji Kawabe, Katsura Takano, Mitsuaki Moriyama, Yoichi Nakamura
Activation of glial cells has been observed in neurodegenerative diseases including Alzheimer's disease (AD). Aggregation of amyloid β (Aβ) is profusely observed as characteristic pathology in AD brain. In our previous study using microglial cell line BV-2, tissue-type transglutaminase (TG2) was found to be involved in phagocytosis (Kawabe et al., in Neuroimmunomodulation 22(4):243-249, 2015; Kawabe et al., Neurochem Res 2017). In the present study, we examined whether TG2 and milk fat globule EGF factor 8 protein (MFG-E8), an adaptor protein promotes macrophage to engulf apoptotic cells, were involved in Aβ endocytosis...
May 3, 2017: Neurochemical Research
https://www.readbyqxmd.com/read/28462876/-aa-amyloidosis
#8
Katia Stankovic Stojanovic, Sophie Georgin-Lavialle, Gilles Grateau
AA amyloidosis remains one of the three main types of systemic amyloidosis with AL and ATTR. Its incidence has been however decreasing recently in Western countries. Chronic inflammatory diseases are currently the first cause of AA amyloidosis, including rheumatoid arthritis, spondyloarthritis and autoinflammatory diseases. Castleman's disease is a specific cause of AA amyloidosis that can be cured by surgery. A chronic inflammatory response is required to develop amyloidosis. Other genetic and environmental factors are also involved...
April 24, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28456755/light-chain-cardiac-amyloidosis-strategies-to-promote-early-diagnosis-and-cardiac-response
#9
REVIEW
Martha Grogan, Angela Dispenzieri, Morie A Gertz
Amyloid light chain (AL) amyloidosis is a systemic disease characterised by the aggregation of misfolded immunoglobulin light chain (LC), predominantly in the heart and kidneys, causing organ failure. If untreated, the median survival of patients with cardiac AL amyloidosis is 6 months from the onset of heart failure. Protracted time to establish a diagnosis, often lasting >1 year, is a frequent factor in poor treatment outcomes. Cardiologists, to whom patients are often referred, frequently miss the opportunity to diagnose cardiac AL amyloidosis...
April 29, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28434353/a-novel-murine-system-for-validating-the-specific-targeting-of-peptides-to-light-chain-associated-al-amyloid
#10
Shannon P Beierle, James S Foster, Tina Richey, Alan Stuckey, Sallie Macy, Stephen J Kennel, Jonathan S Wall
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434330/significant-association-between-renal-function-and-area-of-amyloid-deposition-evident-in-kidney-biopsy-specimens-in-both-aa-and-al-amyloidosis
#11
Takeshi Kuroda, Yumi Ito, Naofumi Imai, Yukiko Nozawa, Hiroe Sato, Takeshi Nakatsue, Yoko Wada, Mitsuhiro Ueno, Masaaki Nakano, Ichiei Narita
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434267/amyloid-in-bone-marrow-smears-in-systemic-light-chain-amyloidosis
#12
Christoph Kimmich, Stefan Schönland, Sandra Kräker, Mindaugas Andrulis, Anthony D Ho, Gudrun Mayer, Tobias Dittrich, Michael Hundemer, Ute Hegenbart
We performed a prospective sensitivity analysis to detect amyloid in bone marrow (BM) smears stained with Congo red (CR) and according to Pappenheim of patients with systemic light-chain (AL) amyloidosis. Results were directly compared to routine BM histology and fat aspiration. We analysed 198 BM smears from patients with the diagnosis or suspicion of systemic AL amyloidosis. Ultimately, the diagnosis could be established for 168 patients. Amyloid was detected on BM smears with CR in 33% (56/168). All patients suspicious for amyloid on Pappenheim staining (n = 39) showed substantial amyloid infiltration on CR...
April 23, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28405187/identification-of-cerebral-metal-ion-imbalance-in-the-brain-of-aging-octodon-degus
#13
Nady Braidy, Anne Poljak, Chris Marjo, Helen Rutlidge, Anne Rich, Bat-Erdene Jugder, Tharusha Jayasena, Nibaldo C Inestrosa, Perminder S Sachdev
The accumulation of redox-active transition metals in the brain and metal dyshomeostasis are thought to be associated with the etiology and pathogenesis of several neurodegenerative diseases, and Alzheimer's disease (AD) in particular. As well, distinct biometal imaging and role of metal uptake transporters are central to understanding AD pathogenesis and aging but remain elusive, due inappropriate detection methods. We therefore hypothesized that Octodon degus develop neuropathological abnormalities in the distribution of redox active biometals, and this effect may be due to alterations in the expression of lysosomal protein, major Fe/Cu transporters, and selected Zn transporters (ZnTs and ZIPs)...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28393574/transbronchial-biopsies-safely-diagnose-amyloid-lung-disease
#14
Praveen Govender, Colleen M Keyes, Elizabeth A Hankinson, Carl J O'Hara, Vaishali Sanchorawala, John L Berk
BACKGROUND: Autopsy identifies lung involvement in 58-92% of patients with the most prevalent forms of systemic amyloidoses. In the absence of lung biopsies, amyloid lung disease often goes unrecognized. Report of a death following transbronchial biopsies in a patient with systemic amyloidosis cautioned against the procedure in this patient cohort. We reviewed our experience with transbronchial biopsies in patients with amyloidosis to determine the safety and utility of bronchoscopic lung biopsies...
April 10, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28392295/oleocanthal-ameliorates-amyloid-%C3%AE-oligomers-toxicity-on-astrocytes-and-neuronal-cells-in-vitro-studies
#15
Yazan S Batarseh, Loqman A Mohamed, Sweilem B Al Rihani, Youssef M Mousa, Abu Bakar Siddique, Khalid A El Sayed, Amal Kaddoumi
Extra-virgin olive oil (EVOO) has several health promoting effects. Evidence have shown that EVOO attenuates the pathology of amyloid-β (Aβ) and improves cognitive function in experimental animal models, suggesting it's potential to protect and reduce the risk of developing Alzheimer's disease (AD). Available studies have linked this beneficial effect to oleocanthal, one of the active components in EVOO. The effect of oleocanthal against AD pathology has been linked to its ability to attenuate Aβ and tau aggregation in vitro, and enhance Aβ clearance from the brains of wild-type and AD transgenic mice in vivo...
June 3, 2017: Neuroscience
https://www.readbyqxmd.com/read/28383130/clinical-characteristics-and-prognostic-factors-in-multiple-myeloma-patients-with-light-chain-deposition-disease
#16
Meera Mohan, Amy Buros, Pankaj Mathur, Neriman Gokden, Manisha Singh, Sandra Susanibar, Jorge Jo Kamimoto, Shadiqul Hoque, Muthukumar Radhakrishnan, Aasiya Matin, Cynthia Davis, Monica Grazziutti, Sharmilan Thanendrarajan, Frits van Rhee, Maurizio Zangari, Faith Davies, Gareth Morgan, Joshua Epstein, Bart Barlogie, Carolina Schinke
Light chain deposition disease (LCDD) is characterized by monotypic immunoglobulin depositions which will eventually lead to loss of organ function if left untreated. While the kidney is almost always affected, the presence and degree of LCDD in other organs vary. Ten to 30% of LCDD patients have underlying Multiple Myeloma (MM), yet outcome and prognostic markers in this particular patient group are still lacking. Here, we analyzed 69 patients with MM and biopsy proven LCDD and report on renal and extra-renal involvement and its impact on prognosis as well as renal response depending on hematologic response...
April 6, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28371615/mechanisms-that-synergistically-regulate-%C3%AE-secretase-processing-of-app-and-a%C3%AE-%C3%AE-protein-levels-relevance-to-pathogenesis-and-treatment-of-alzheimer-s-disease
#17
Joseph Ward, Haizhi Wang, Aleister J Saunders, Rudolph E Tanzi, Can Zhang
The pathophysiology of Alzheimer's disease (AD) is characterized by the formation of cerebral β-amyloid plaque from a small peptide amyloid-β (Aβ). Aβ is generated from the canonical amyloid-β precursor protein (APP) proteolysis pathway through β- and γ-secretases. Decreasing Aβ levels through targeting APP processing is a very promising direction in clinical trials for AD. A novel APP processing pathway was recently identified, in which η-secretase processing of APP occurs and results in the generation of the carboxy-terminal fragment-η (CTF-η or η-CTF) (Wang et al...
February 2017: Discovery Medicine
https://www.readbyqxmd.com/read/28356893/alzheimer-s-toxic-amyloid-beta-oligomers-unwelcome-visitors-to-the-na-k-atpase-alpha3-docking-station
#18
Thomas DiChiara, Nadia DiNunno, Jeffrey Clark, Riana Lo Bu, Erika N Cline, Madeline G Rollins, Yuesong Gong, David L Brody, Stephen G Sligar, Pauline T Velasco, Kirsten L Viola, William L Klein
Toxic amyloid beta oligomers (AβOs) are known to accumulate in Alzheimer's disease (AD) and in animal models of AD. Their structure is heterogeneous, and they are found in both intracellular and extracellular milieu. When given to CNS cultures or injected ICV into non-human primates and other non-transgenic animals, AβOs have been found to cause impaired synaptic plasticity, loss of memory function, tau hyperphosphorylation and tangle formation, synapse elimination, oxidative and ER stress, inflammatory microglial activation, and selective nerve cell death...
March 2017: Yale Journal of Biology and Medicine
https://www.readbyqxmd.com/read/28350808/differential-recruitment-efficacy-of-patient-derived-amyloidogenic-and-myeloma-light-chain-proteins-by-synthetic-fibrils-a-metric-for-predicting-amyloid-propensity
#19
Emily B Martin, Angela Williams, Craig Wooliver, R Eric Heidel, Sarah Adams, John Dunlap, Marina Ramirez-Alvarado, Luis M Blancas-Mejia, Ronald H Lands, Stephen J Kennel, Jonathan S Wall
BACKGROUND: Monoclonal free light chain (LC) proteins are present in the circulation of patients with immunoproliferative disorders such as light chain (AL) amyloidosis and multiple myeloma (MM). Light chain-associated amyloid is a complex pathology composed of proteinaceous fibrils and extracellular matrix proteins found in all patients with AL and in ~10-30% of patients who presented with MM. Amyloid deposits systemically in multiple organs and tissues leading to dysfunction and ultimately death...
2017: PloS One
https://www.readbyqxmd.com/read/28341732/novel-recurrent-chromosomal-aberrations-detected-in-clonal-plasma-cells-of-light-chain-amyloidosis-patients-show-potential-adverse-prognostic-effect-first-results-from-a-genome-wide-copy-number-array-analysis
#20
Martin Granzow, Ute Hegenbart, Katrin Hinderhofer, Dirk Hose, Anja Seckinger, Tilmann Bochtler, Kari Hemminki, Hartmut Goldschmidt, Stefan O Schönland, Anna Jauch
Immunoglobulin light chain amyloidosis is a rare plasma cell dyscrasia characterized by deposition of abnormal amyloid fibrils in multiple organs impairing their function. In the largest cohort studied up to now of 118 CD138-purified plasma cell samples from previously untreated immunoglobulin light chain amyloidosis patients, we assessed in parallel copy number alterations using high-density copy number arrays and interphase fluorescence in situ hybridization. We used fluorescence in situ hybridization probes for the IgH translocations t(11;14), t(4;14), and t(14;16) or any other IgH rearrangement as well as numerical aberrations of the chromosome loci 1q21, 8p21, 5p15/5q35, 11q22...
March 24, 2017: Haematologica
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