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https://www.readbyqxmd.com/read/28739313/cardiac-amyloidosis-an-update-on-pathophysiology-diagnosis-and-treatment
#1
REVIEW
Omar K Siddiqi, Frederick L Ruberg
The amyloidoses are a group of systemic diseases characterized by organ deposition of misfolded protein fragments of diverse origins. The natural history of the disease, involvement of other organs, and treatment options vary significantly based on the protein of origin. In AL amyloidosis, amyloid protein is derived from immunoglobulin light chains, and most often involves the kidneys and the heart. ATTR amyloidosis is categorized as mutant or wild-type depending on the genetic sequence of the transthyretin (TTR) protein produced by the liver...
July 13, 2017: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28736891/heat-induced-native-dimerization-prevents-amyloid-formation-by-variable-domain-from-immunoglobulin-light-chain-rei
#2
Marina Nawata, Hirotaka Tsutsumi, Yuta Kobayashi, Satoru Unzai, Shouhei Mine, Tsutomu Nakamura, Koichi Uegaki, Hironari Kamikubo, Mikio Kataoka, Daizo Hamada
Amyloid light chain (AL) amyloidosis is a protein-misfolding disease characterized by accumulation of immunoglobulin light chains (LCs) into amyloid fibrils. Dimerization of full length or variable domain (VL ) of LC serves to stabilize the native state and prevent the formation of amyloid fibrils. We here analyzed the thermodynamic properties of dimerization and unfolding reactions by nonamyloidogenic VL from REI LC or its monomeric Y96K mutant using sedimentation velocity and circular dichroism. The data indicate that the equilibrium shifts to native dimerization for wild type REI VL by elevating temperature due to the negative enthalpy change for dimer dissociation (-81...
July 24, 2017: FEBS Journal
https://www.readbyqxmd.com/read/28736850/progressive-ataxia-and-palatal-tremor-two-autopsy-cases-of-a-novel-tauopathy
#3
Andrew F Gao, Achinoam Faust-Socher, Maryam Al-Murshed, Marc R Del Bigio, Anthony E Lang, David G Munoz
BACKGROUND: Sporadic progressive ataxia and palatal tremor is a rare syndrome characterized by mid- to late-adult-onset symptomatic palatal tremor and slowly progressive cerebellar ataxia. To date, there has been only one autopsy report, which described a novel 4-repeat tauopathy with hypertrophic olivary degeneration and tau-positive inclusions in olivary neurons and dystrophic neuritic processes termed glomeruloid bodies. We report on 2 additional autopsy cases. METHODS: Sections from selected paraffin-embedded brain regions were stained with hematoxylin and eosin/Luxol fast blue and processed for phosphorylated tau, 3-repeat tau, 4-repeat tau, neurofilament, glial fibrillary acid protein, phosphorylated α-synuclein, phosphorylated TAR DNA-binding protein 43, beta-amyloid, and p62 immunohistochemistry...
July 24, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28729034/prolonged-renal-survival-in-light-chain-amyloidosis-speed-and-magnitude-of-light-chain-reduction-is-the-crucial-factor
#4
Tamer Rezk, Helen J Lachmann, Marianna Fontana, Sajitha Sachchithanantham, Shameem Mahmood, Aviva Petrie, Carol J Whelan, Jennifer H Pinney, Darren Foard, Thirusha Lane, Taryn Youngstein, Ashutosh D Wechalekar, Paul Bass, Philip N Hawkins, Julian D Gillmore
Renal involvement causing progressive chronic kidney disease (CKD) is present in 70% of patients with systemic Ig light-chain (AL) amyloidosis at diagnosis. Chemotherapy that substantially suppresses free light chain production is associated with improved patient survival, but its benefit in delaying the onset of renal replacement therapy among patients who present with established advanced CKD has not been studied. To evaluate this, we studied 1000 patients enrolled in the prospective UK AL amyloidosis chemotherapy study (ALchemy)...
July 17, 2017: Kidney International
https://www.readbyqxmd.com/read/28728692/magnetic-resonance-in-transthyretin%C3%A2-cardiac-amyloidosis
#5
Ana Martinez-Naharro, Thomas A Treibel, Amna Abdel-Gadir, Heerajnarain Bulluck, Giulia Zumbo, Daniel S Knight, Tushar Kotecha, Rohin Francis, David F Hutt, Tamer Rezk, Stefania Rosmini, Candida C Quarta, Carol J Whelan, Peter Kellman, Julian D Gillmore, James C Moon, Philip N Hawkins, Marianna Fontana
BACKGROUND: Cardiac transthyretin amyloidosis (ATTR) is an increasingly recognized cause of heart failure. Cardiac magnetic resonance (CMR), with late gadolinium enhancement (LGE) and T1 mapping, is emerging as a reference standard for diagnosis and characterization of cardiac amyloidosis. OBJECTIVES: The authors used CMR with extracellular volume fraction (ECV) measurement to characterize cardiac involvement in relation to outcome in ATTR. METHODS: Subjects comprised 263 patients with cardiac ATTR corroborated by grade 2 to 3 (99m)Tc-DPD ((99m)Tc-3,3-diphosphono-1,2-propanodicarboxylic acid) cardiac uptake, 17 with suspected cardiac ATTR (grade 1 (99m)Tc-DPD), and 12 asymptomatic individuals with amyloidogenic transthyretin (TTR) mutations...
July 25, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28722055/the-amyloid-architecture-provides-a-scaffold-for-enzyme-like-catalysts
#6
Z S Al-Garawi, B A McIntosh, D Neill-Hall, A A Hatimy, S M Sweet, M C Bagley, L C Serpell
Natural biological enzymes possess catalytic sites that are generally surrounded by a large three-dimensional scaffold. However, the proportion of the protein molecule that participates in the catalytic reaction is relatively small. The generation of artificial or miniature enzymes has long been a focus of research because enzyme mimetics can be produced with high activity at low cost. These enzymes aim to mimic the active sites without the additional architecture contributed by the protein chain. Previous work has shown that amyloidogenic peptides are able to self-assemble to create an active site that is capable of binding zinc and catalysing an esterase reaction...
July 19, 2017: Nanoscale
https://www.readbyqxmd.com/read/28719622/characterization-of-plasma-metal-profiles-in-alzheimer-s-disease-using-multivariate-statistical-analysis
#7
Chunmei Guan, Rui Dang, Yu Cui, Liyan Liu, Xiaobei Chen, Xiaoyu Wang, Jingli Zhu, Donggang Li, Junwei Li, Decai Wang
The exact cause of Alzheimer's disease (AD) and the role of metals in its etiology remain unclear. We have used an analytical approach, based on inductively coupled plasma mass spectrometry coupled with multivariate statistical analysis, to study the profiles of a wide range of metals in AD patients and healthy controls. AD cannot be cured and the lack of sensitive biomarkers that can be used in the early stages of the disease may contribute to this treatment failure. In the present study, we measured plasma levels of amyloid-β1-42(0...
2017: PloS One
https://www.readbyqxmd.com/read/28705053/cardiac-amyloidosis-pathogenesis-clinical-context-diagnosis-and-management-options
#8
Hassan Alkhawam, Darshan Patel, James Nguyen, Saumya Mariam Easaw, Mohammed Al-Sadawi, Umer Syed, Feras Zaiem, Maher Homsi, Timothy J Vittorio
Amyloidosis covers a group of disorders that can manifest in virtually any organ system in the body and is thought to be secondary to misfolding of extracellular proteins with subsequent deposition in tissues. The precursor protein that is produced in excess defines the specific amyloid type. This requires histopathological confirmation using Congo-red dye with its characteristic demonstration of green birefringence under cross-polarized light. There are three main types of amyloidosis associated with cardiac involvement: light-chain (AL), familial or senile (ATTR), and secondary (AA) amyloidosis...
July 14, 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28700676/imaging-features-of-intracerebral-hemorrhage-with-cerebral-amyloid-angiopathy-systematic-review-and-meta-analysis
#9
Neshika Samarasekera, Mark Alexander Rodrigues, Pheng Shiew Toh, Rustam Al-Shahi Salman
BACKGROUND: We sought to summarize Computed Tomography (CT)/Magnetic Resonance Imaging (MRI) features of intracerebral hemorrhage (ICH) associated with cerebral amyloid angiopathy (CAA) in published observational radio-pathological studies. METHODS: In November 2016, two authors searched OVID Medline (1946-), Embase (1974-) and relevant bibliographies for studies of imaging features of lobar or cerebellar ICH with pathologically proven CAA ("CAA-associated ICH")...
2017: PloS One
https://www.readbyqxmd.com/read/28679987/analysis-of-deposited-peptides-in-amyloid-lesions-of-al-amyloidosis-by-mass-spectrometry
#10
Toshiya Sugimoto, Masayoshi Tasaki, Taro Yamashita, Mitsuharu Ueda, Yukio Ando, Konen Obayashi, Yutaka Okuno, Hiroyuki Hata
Although immunoglobulin light chain (LC) plays a critical role in AL amyloidosis, detailed characteristics of deposited LC are not well known. In this study, LC peptides were analyzed by a combination of laser microdissection, liquid chromatography, and mass spectrometry (LC-MS/MS) in 65 patients with AL amyloidosis. Constant regions of LC were detected in all patients. Kappa- or lambda-types were detected in 20 and 45 patients, respectively. Various types of constant regions of LC were found; however, IGLC3 and IGKC4 were the most frequently detected...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28672189/multi-scale-modeling-of-altered-synaptic-plasticity-related-to-amyloid-%C3%AE-effects
#11
Takumi Matsuzawa, László Zalányi, Tamás Kiss, Péter Érdi
As suggested by Palop and Mucke (2010) pathologically elevated β-amyloid (Aβ) impairs long term potentiation (LTP) and enhances long term depression (LTD) possible underlying mechanisms in Alzheimer's Disease (AD). In the present paper we adopt and further elaborate a phenomenological computational model of bidirectional plasticity based on the calcium control hypothesis of Shouval et al. (2002). First, to account for Aβ effects the activation function Ω was modified assuming competition between LTP and LTD, and parameter sets were identified that well describe both normal and pathological synaptic plasticity processes...
June 20, 2017: Neural Networks: the Official Journal of the International Neural Network Society
https://www.readbyqxmd.com/read/28647708/relapsing-cerebral-amyloid-angiopathy-related-inflammation-the-wax-and-the-wane
#12
Sharfaraz Salam, Mayooreshan Anandarajah, Sarah Al-Bachari, Piyali Pal, Jonathan Sussman, Hisham Hamdalla
Cerebral amyloid angiopathy-related inflammation (CAA-I) is a rare variant of cerebral amyloid angiopathy (CAA). Its precise pathophysiology remains uncertain and we currently have limited evidence on which immunosuppressive agents are the most effective in its treatment. The disease course of CAA-I disorders can vary from an isolated clinical event to recurrent episodes. We present a case of biopsy-confirmed CAA-I that gives insight into its potential relapsing nature and the challenges of its long-term management...
June 24, 2017: Practical Neurology
https://www.readbyqxmd.com/read/28647518/increasing-the-accuracy-of-proteomic-typing-by-decellularisation-of-amyloid-tissue-biopsies
#13
P Patrizia Mangione, Giuseppe Mazza, Janet A Gilbertson, Nigel B Rendell, Diana Canetti, Sofia Giorgetti, Luca Frenguelli, Marco Curti, Tamer Rezk, Sara Raimondi, Mark B Pepys, Philip N Hawkins, Julian D Gillmore, Graham W Taylor, Massimo Pinzani, Vittorio Bellotti
Diagnosis and treatment of systemic amyloidosis depend on accurate identification of the specific amyloid fibril protein forming the tissue deposits. Confirmation of monoclonal immunoglobulin light chain amyloidosis (AL), requiring cytotoxic chemotherapy, and avoidance of such treatment in non-AL amyloidosis, are particularly important. Proteomic analysis characterises amyloid proteins directly. It complements immunohistochemical staining of amyloid to identify fibril proteins and gene sequencing to identify mutations in the fibril precursors...
June 21, 2017: Journal of Proteomics
https://www.readbyqxmd.com/read/28642699/cerebral-blood-flow-and-amyloid-%C3%AE-interact-to-affect-memory-performance-in-cognitively-normal-older-adults
#14
Katherine J Bangen, Alexandra L Clark, Emily C Edmonds, Nicole D Evangelista, Madeleine L Werhane, Kelsey R Thomas, Lyzette E Locano, My Tran, Zvinka Z Zlatar, Daniel A Nation, Mark W Bondi, Lisa Delano-Wood
Cerebral blood flow (CBF) alterations and amyloid-β (Aβ) accumulation have been independently linked to cognitive deficits in older adults at risk for dementia. Less is known about how CBF and Aβ may interact to affect cognition in cognitively normal older adults. Therefore, we examined potential statistical interactions between CBF and Aβ status in regions typically affected in Alzheimer's disease (AD) within a sample of older adults from the Alzheimer's Disease Neuroimaging Initiative (ADNI) study. Sixty-two cognitively normal participants (mean age = 72 years) underwent neuroimaging and memory testing...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28637305/left-atrial-structure-and-function-in-cardiac-amyloidosis
#15
Kotaro Nochioka, Candida Cristina Quarta, Brian Claggett, Gabriela Querejeta Roca, Claudio Rapezzi, Rodney H Falk, Scott D Solomon
Aims: Although cardiac amyloidosis (CA) is characterized by significant left atrial (LA) dilatation, the characteristics of LA function remain to be fully investigated. Methods and results: We assessed LA function by speckle-tracking echocardiography in 124 patients with CA and sinus rhythm: 68 with light chain (AL), 29 with mutant (ATTRm), 27 with wild-type (ATTRwt) transthyretin amyloidosis. Conventional and strain-derived parameters, including LA peak longitudinal strain (LS) and strain rate (peak LSR: reservoir function; early LSR: conduit function; late LSR: active function), were assessed compared between CA patients and 20 healthy controls of similar age and gender...
June 16, 2017: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28632471/lenalidomide-as-a-treatment-for-relapsed-al-amyloidosis-in-an-hiv-positive-patient
#16
Johanna Denman, Kaveh Manavi, Mark Cook
We present a case of an HIV-positive man with systemic immunoglobulin light chain (AL) amyloid with cardiac involvement. At relapse, he was treated with lenalidomide and dexamethasone having previously developed autonomic neuropathy with bortezomib-based chemotherapy. The patient achieved a serological complete response with symptomatic improvement. After 11 cycles, lenalidomide was discontinued due to extensive ischaemia of the gastrointestinal tract. The patient remains symptomatically stable with normal levels of serum-free light chains 11 months after the treatment was discontinued...
January 1, 2017: International Journal of STD & AIDS
https://www.readbyqxmd.com/read/28632419/in-vitro-co-expression-of-human-amyloidogenic-immunoglobulin-light-and-heavy-chain-proteins-a-relevant-cell-based-model-of-al-amyloidosis
#17
Elena S Klimtchuk, Tatiana B Prokaeva, Brian H Spencer, Olga Gursky, Lawreen H Connors
Immunoglobulin (Ig) light chain (LC) amyloidosis (AL) is characterized by the overproduction and tissue deposition of monoclonal LC in various organs and tissues. The plasma circulating monoclonal LC is believed to be the precursor of the deposited protein and in vitro studies aimed at understanding AL pathobiology have mainly focused on LC and its variable domain. While 33% of patients have free circulating monoclonal LC, ∼40% feature LC complexed to heavy chain (HC) forming a monoclonal intact Ig; the significance of free vs...
June 20, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28630445/myocardial-deformation-in-cardiac-amyloid-light-chain-amyloidosis-assessed-with-3t-cardiovascular-magnetic-resonance-feature-tracking
#18
Rui Li, Zhi-Gang Yang, Hua-Yan Xu, Ke Shi, Xi Liu, Kai-Yue Diao, Ying-Kun Guo
Clinically, assessment of myocardial function is essential in patients with amyloid light-chain cardiac amyloidosis (AL-CA) to predict outcome and determine therapeutic approach. The aim of this study was to investigate the feasibility of cardiovascular magnetic resonance (CMR)-derived feature tracking algorithm for assessing left ventricular (LV) myocardial deformation in AL-CA, and to determine if these abnormal myocardial deformation parameters are correlated to impaired LV myocardial microvascular dysfunction...
June 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28622303/addition-of-cyclophosphamide-and-higher-doses-of-dexamethasone-do-not-improve-outcomes-of-patients-with-al-amyloidosis-treated-with-bortezomib
#19
E Kastritis, M Gavriatopoulou, M Roussou, D Fotiou, D C Ziogas, M Migkou, E Eleutherakis-Papaiakovou, I Panagiotidis, N Kanellias, E Psimenou, E Papadopoulou, C Pamboucas, E Manios, H Gakiopoulou, A Ntalianis, A Tasidou, S Giannouli, E Terpos, M A Dimopoulos
Bortezomib, in combination with dexamethasone (VD) or with the addition of cyclophosphamide (VCD), is highly effective in patients with amyloid light-chain (AL) amyloidosis. Currently, VCD is considered as a primary regimen for patients with AL, but it is not clear whether the addition of cyclophosphamide to VD further and significantly improves efficacy, given the substantial activity of bortezomib itself. We retrospectively compared the outcomes of 101 patients with AL amyloidosis who received VD (n=59) or VCD (n=42) in two consecutive periods...
June 16, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/28618364/amyloid-like-protein-nanofibrous-membranes-as-a-sensing-layer-infrastructure-for-the-design-of-mass-sensitive-biosensors
#20
Gözde Kabay, Gizem Kaleli Can, Mehmet Mutlu
Quartz crystal microbalances (QCMs) have been used in the literature for mass sensitive biosensor applications. However, their performance, reliability and stability have been limited by the chemical treatment steps required for the functionalization and activation of the QCM surface, prior to antibody immobilization. Specifically, these steps cause increased film thickness, which diminishes performance by mass overload, and create a harsh environment, which reduces biological activity. In this work, we eliminated this chemical step by introducing a sensing layer modification using electrospun amyloid like-bovine serum albumin (AL-BSA) nanofibers on QCM surfaces...
June 10, 2017: Biosensors & Bioelectronics
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