keyword
MENU ▼
Read by QxMD icon Read
search

Al amyloid

keyword
https://www.readbyqxmd.com/read/29331631/the-edinburgh-ct-and-genetic-diagnostic-criteria-for-lobar-intracerebral-haemorrhage-associated-with-cerebral-amyloid-angiopathy-model-development-and-diagnostic-test-accuracy-study
#1
Mark A Rodrigues, Neshika Samarasekera, Christine Lerpiniere, Catherine Humphreys, Mark O McCarron, Philip M White, James A R Nicoll, Cathie L M Sudlow, Charlotte Cordonnier, Joanna M Wardlaw, Colin Smith, Rustam Al-Shahi Salman
BACKGROUND: Identification of lobar spontaneous intracerebral haemorrhage associated with cerebral amyloid angiopathy (CAA) is important because it is associated with a higher risk of recurrent intracerebral haemorrhage than arteriolosclerosis-associated intracerebral haemorrhage. We aimed to develop a prediction model for the identification of CAA-associated lobar intracerebral haemorrhage using CT features and genotype. METHODS: We identified adults with first-ever intracerebral haemorrhage diagnosed by CT, who died and underwent research autopsy as part of the Lothian IntraCerebral Haemorrhage, Pathology, Imaging and Neurological Outcome (LINCHPIN) study, a prospective, population-based, inception cohort...
January 10, 2018: Lancet Neurology
https://www.readbyqxmd.com/read/29317708/connexin-43-hemichannel-as-a-novel-mediator-of-sterile-and-infectious-inflammatory-diseases
#2
Wei Li, Guoqiang Bao, Weiqiang Chen, Xiaoling Qiang, Shu Zhu, Shuaiwei Wang, Mingzhu He, Gaifeng Ma, Mahendar Ochani, Yousef Al-Abed, Huan Yang, Kevin J Tracey, Ping Wang, John D'Angelo, Haichao Wang
Cytoplasmic membrane-bound connexin 43 (Cx43) proteins oligomerize into hexameric channels (hemichannels) that can sometimes dock with hemichannels on adjacent cells to form gap junctional (GJ) channels. However, the possible role of Cx43 hemichannels in sterile and infectious inflammatory diseases has not been adequately defined due to the lack of selective interventions. Here we report that a proinflammatory mediator, the serum amyloid A (SAA), resembled bacterial endotoxin by stimulating macrophages to up-regulate Cx43 expression and double-stranded RNA-activated protein kinase R (PKR) phosphorylation in a TLR4-dependent fashion...
January 9, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29303069/unfolding-cardiac-amyloidosis-from-pathophysiology-to-cure
#3
Klemens Ablasser, Nicolas Verheyen, Theresa Glantschnig, Giulio Agnetti, Peter P Rainer
Deposition of amyloidogenic proteins leading to the formation of amyloid fibrils in the myocardium cause cardiac amyloidosis. Although any form of systemic amyloidosis can affect the heart, light-chain (AL) or transthyretin amyloidosis (ATTR) account for the majority of diagnosed cardiac amyloid deposition. The extent of cardiac disease independently predicts mortality. The reversal or arrest of adverse cardiac remodeling is the target of current therapies, as cardiac-related mortality worsens prognosis in patients where the underlying systemic amyloidosis was successfully treated...
January 4, 2018: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/29282382/massive-upper-and-lower-gi-bleed-from-simultaneous-primary-al-amyloidosis-of-the-stomach-and-transverse-colon-in-a-patient-with-multiple-myeloma
#4
Zubair Khan, Umar Darr, Anas Renno, Abhinav Tiwari, Aijaz Sofi, Ali Nawras
Symptomatic primary (amyloid light-chain or AL) amyloidosis of the gastrointestinal (GI) tract is very rare. Most of the patients with symptomatic involvement of the GI tract present with altered motility, malabsorption, or bleeding. We report a case of gastric and colonic amyloidosis on anticoagulation presenting with massive upper and lower GI bleeding. A 67-year-old lady known to have multiple myeloma and AL amyloidosis on rivaroxaban presented with massive upper GI bleeding. Esophagogastroduodenoscopy showed a mass lesion (3 × 7 cm) located along the greater curvature in the body/antrum with active bleeding...
September 2017: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/29247143/cognitive-impairment-before-intracerebral-hemorrhage-is-associated-with-cerebral-amyloid-angiopathy
#5
Gargi Banerjee, Duncan Wilson, Gareth Ambler, Karen Osei-Bonsu Appiah, Clare Shakeshaft, Surabhika Lunawat, Hannah Cohen, Tarek Yousry, Gregory Y H Lip, Keith W Muir, Martin M Brown, Rustam Al-Shahi Salman, Hans Rolf Jäger, David J Werring
BACKGROUND AND PURPOSE: Although the association between cerebral amyloid angiopathy (CAA) and cognitive impairment is increasingly recognized, it is not clear whether this is because of the impact of recurrent intracerebral hemorrhage (ICH) events, disruptions caused by cerebral small vessel damage, or both. We investigated this by considering whether cognitive impairment before ICH was associated with neuroimaging features of CAA on magnetic resonance imaging. METHODS: We studied 166 patients with neuroimaging-confirmed ICH recruited to a prospective multicentre observational study...
December 15, 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/29232098/amyloid-%C3%AE-1-40-toxicity-depends-on-the-molecular-contact-between-phenylalanine-19-and-leucine-34
#6
Alexander Korn, Steffane McLennan, Juliane Adler, Martin Krueger, Dayana Surendran, Sudipta Maiti, Daniel Huster
The formation of the hydrophobic contact between phenylalanine 19 (F19) and leucine 34 (L34) of amyloid β (1-40) (Aβ(1-40)) is known to be an important step in the fibrillation of Aβ(1-40) peptides. Mutations of this putatively early molecular contact were shown to strongly influence the toxicity of Aβ(1-40) (Das et al., ACS Chem. Neurosci. 6 (2015) 1290-1295). Any mutation of residue F19 completely abolished the toxicity of Aβ(1-40), suggesting that a proper F19 - L34 contact is crucial also for the formation of transient oligomers...
December 12, 2017: ACS Chemical Neuroscience
https://www.readbyqxmd.com/read/29225107/comparison-of-blood-brain-barrier-penetration-efficiencies-between-linear-and-cyclic-all-d-enantiomeric-peptides-developed-for-the-treatment-of-alzheimer-s-disease
#7
Elena Schartmann, Sarah Schemmert, Tamar Ziehm, Leonie H E Leithold, Nan Jiang, Markus Tusche, N Joni Shah, Karl-Josef Langen, Janine Kutzsche, Dieter Willbold, Antje Willuweit
Alzheimer's disease (AD), until now, is an incurable progressive neurodegenerative disease. To target toxic amyloid β oligomers in AD patients' brains and to convert them into non-toxic aggregation-incompetent species, we designed peptides consisting solely of d-enantiomeric amino acid residues. The original lead compound was named D3 and several D3 derivatives were designed to enhance beneficial properties. Here, we compare four d-peptides concerning their efficiencies to pass the blood-brain barrier (BBB)...
December 7, 2017: European Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/29222812/spheroid-type-of-al-amyloid-deposition-associated-with-colonic-adenocarcinoma-a-case-report-with-literature-review
#8
Moon Joo Kim, Zulfia McCroskey, Yingchao Piao, Anna Belcheva, Luan Truong, Paul J Kurtin, Jae Y Ro
We report a colonic adenocarcinoma associated with diffuse submucosal deposition of a peculiar spheroid-type amyloid identified in the colon, terminal ileum, and appendix. A 65-year-old woman with past medical histories of hypertension, and chronic obstructive pulmonary disease, presented to the emergency room with cramping abdominal pain and nausea. A computed tomography (CT) scan of abdomen showed right colonic volvulus. Emergency right hemicolectomy was performed. The specimen showed colonic adenocarcinoma with focal submucosal invasion (pT1) arising from a villotubular adenoma...
December 8, 2017: Pathology International
https://www.readbyqxmd.com/read/29222231/al-amyloidosis-from-molecular-mechanisms-to-targeted-therapies
#9
REVIEW
Giampaolo Merlini
Systemic amyloidosis is caused by misfolding and extracellular deposition of circulating proteins as amyloid fibrils, resulting in the dysfunction of vital organs. The most common systemic amyloidosis, light-chain (AL) amyloidosis, is caused by misfolded light chains produced by a small, dangerous B-cell clone. The process of amyloid formation, organ targeting, and damage is multifaceted and, after disease initiation, the complexity of the downstream pathogenic cascade increases, rendering its control a challenge...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29217108/improved-outcomes-after-heart-transplantation-for-cardiac-amyloidosis-in-the-modern-era
#10
Arnt V Kristen, Michael M Kreusser, Patrick Blum, Stefan O Schönland, Lutz Frankenstein, Andreas O Dösch, Benjamin Knop, Matthias Helmschrott, Bastian Schmack, Arjang Ruhparwar, Ute Hegenbart, Hugo A Katus, Philip W J Raake
BACKGROUND: Cardiac amyloidosis, caused most commonly by deposition of light chain (AL) or transthyretin (ATTR) type fibrils, has an extremely poor prognosis. In this retrospective single-center study, we evaluated temporal trends in survival after heart transplantation for cardiac amyloidosis. METHODS: We analyzed 48 patients with cardiac amyloidosis (AL, n = 32; familial ATTR, n = 16) who underwent heart transplantation from May 2002 to March 2017. Patients were analysed in 2 periods, Era 1 (2002- 2007) and Era 2 (2008- 2017), separated by altered patient selection in both, AL and ATTR amyloidosis, and changed chemotherapy regimens for AL amyloidosis...
November 15, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29212103/recurrent-diffuse-gastric-bleeding-as-a-leading-symptom-of%C3%A2-gastrointestinal-al-amyloidosis
#11
Caspar Franck, Marino Venerito, Jochen Weigt, Albert Roessner, Peter Malfertheiner
Amyloidosis is a rare disease (incidence about 0.8/100 000) characterized by extracellular tissue deposition of fibrils composed of low molecular weight subunits of a variety of serum proteins. Clinical manifestations are largely determined by the type of precursor protein, the tissue distribution and the amount of amyloid deposition. Gastrointestinal (GI) manifestations of amyloidosis are even more uncommon (3 % of all amyloidosis patients). Symptoms of GI amyloidosis are nonspecific, heterogeneous, and include weight loss, GI bleeding, heartburn, early satiety, diarrhea and abdominal pain...
December 2017: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/29201005/high-concentrations-of-rosiglitazone-reduce-mrna-and-protein-levels-of-lrp1-in-hepg2-cells
#12
Alejandro N Rondón-Ortiz, Christian L Lino Cardenas, Jimena Martínez-Málaga, Ana L Gonzales-Urday, Kuljeet S Gugnani, Mark Böhlke, Timothy J Maher, Alejandro J Pino-Figueroa
Low-density lipoprotein receptor-related protein 1 (LRP1) is an endocytic receptor involved in the uptake of a variety of molecules, such as apoE, α2-macroglobulin, and the amyloid β peptide (Aβ), for either transcellular transport, protein trafficking or lysosomal degradation. The LRP1 gene can be transcribed upon activation of peroxisome proliferator receptor activated-γ (PPARγ) by the potent PPARγ agonist, rosiglitazone (RGZ). In previous studies, RGZ was shown to upregulate LRP1 levels in concentrations between 0...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/29200282/incomplete-refolding-of-antibody-light-chains-to-non-native-protease-sensitive-conformations-leads-to-aggregation-a-mechanism-of-amyloidogenesis-in-patients
#13
Gareth J Morgan, Grace A Usher, Jeffery W Kelly
Genetic, biochemical, and pharmacologic evidence supports the hypothesis that conformationally altered or misfolded protein states enable aggregation and cytotoxicity in the systemic amyloid diseases. Reversible structural fluctuations of natively folded proteins are involved in the aggregation of many degenerative disease associated proteins. Herein, we use antibody light chains (LCs) that form amyloid fibrils in AL amyloidosis to consider an alternative hypothesis of amyloidogenesis: that transient unfolding and incomplete extracellular refolding of secreted proteins can lead to metastable, alternatively folded states that are more susceptible to aggregation or to endoproteolysis that can release aggregation-prone fragments...
December 19, 2017: Biochemistry
https://www.readbyqxmd.com/read/29197423/amnestic-mci-patients-experiences-after-disclosure-of-their-amyloid-pet-result-in-a-research-context
#14
Gwendolien Vanderschaeghe, Jolien Schaeverbeke, Rose Bruffaerts, Rik Vandenberghe, Kris Dierickx
BACKGROUND: Biomarkers such as amyloid imaging are increasingly used for diagnosis in the early stages of Alzheimer's disease. Very few studies have examined this from the perspective of the patient. To date, there is only limited evidence about how patients experience and value disclosure in an early disease stage. METHODS: Semistructured interviews were carried out with 38 patients with amnestic mild cognitive impairment as part of an investigator-driven diagnostic trial (EudraCT, 2013-004671-12; registered on 20 June 2014) in which participants could opt to know the binary outcome (positive/negative) result of their amyloid positron emission tomography (PET) scan...
December 2, 2017: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/29196671/concurrent-structural-and-biophysical-traits-link-with-immunoglobulin-light-chains-amyloid-propensity
#15
Luca Oberti, Paola Rognoni, Alberto Barbiroli, Francesca Lavatelli, Rosaria Russo, Martina Maritan, Giovanni Palladini, Martino Bolognesi, Giampaolo Merlini, Stefano Ricagno
Light chain amyloidosis (AL), the most common systemic amyloidosis, is caused by the overproduction and the aggregation of monoclonal immunoglobulin light chains (LC) in target organs. Due to genetic rearrangement and somatic hypermutation, virtually, each AL patient presents a different amyloidogenic LC. Because of such complexity, the fine molecular determinants of LC aggregation propensity and proteotoxicity are, to date, unclear; significantly, their decoding requires investigating large sets of cases. Aiming to achieve generalizable observations, we systematically characterised a pool of thirteen sequence-diverse full length LCs...
December 1, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29176541/diffuse-peritoneal-and-bowel-wall-infiltration-by-light-chain-al-amyloidosis-with-omental-calcification-mimicking-abdominal-carcinomatosis-an-elderly-female-with-incidental-finding-of-light-chain-monoclonal-gammopathy-of-undetermined-significance-lc-mgus
#16
Shoaib Junejo, Yasir Ali, Sandeep Singh Lubana, Sandeep S Tuli
BACKGROUND Amyloidosis is the extracellular tissue deposition of plasma proteins, which after conformational changes, forms antiparallel beta pleated sheets of fibrils. Amyloid light-chain (AL) is a type of amyloidosis that is due to deposition of proteins derived from immunoglobulin (Ig) light chains. Gastrointestinal tract (GIT) involvement most often found in amyloid A (AA) amyloidosis type. There have been no reports of obstructive GIT AL amyloid patients having monoclonal gammopathy of undetermined significance (MGUS)...
November 25, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/29168166/new-tool-to-tackle-alzheimer-s-disease-amyloid-%C3%AE-protofibril-selective-antibody-absl-an-editorial-highlight-for-the-conformational-epitope-for-a-new-a%C3%AE-42-protofibril-selective-antibody-partially-overlaps-with-the-peptide-n-terminal-region-on-doi-10-1111-jnc
#17
EDITORIAL
Elek Molnár
This Editorial highlights a study by Colvin et al. (2017) in the current issue of Journal of Neurochemistry, in which the authors describe the development and characterisation of a new rabbit antibody (termed antibody St. Louis; AbSL) that preferentially recognises amyloid-β (Aβ) protein 42 (Aβ42) protofibrils over other Aβ species.
November 23, 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/29153741/left-atrial-function-in-patients-with-light-chain-amyloidosis-a-transthoracic-3d-speckle-tracking-imaging-study
#18
Dania Mohty, Vincent Petitalot, Julien Magne, Bahaa M Fadel, Cyrille Boulogne, Dounia Rouabhia, Chahrazed ElHamel, David Lavergne, Thibaud Damy, Victor Aboyans, Arnaud Jaccard
BACKGROUND: Systemic light chain amyloidosis (AL) is characterized by the extracellular deposition of amyloid fibrils. Transthoracic echocardiography is the modality of choice to assess cardiac function in patients with AL. Whereas left ventricular (LV) function has been well studied in this patient population, data regarding the value of left atrial (LA) function in AL patients are lacking. In this study, we aim to examine the impact of LA volumes and function on survival in AL patients as assessed by real-time 3D echocardiography...
November 16, 2017: Journal of Cardiology
https://www.readbyqxmd.com/read/29147557/the-diversity-and-utility-of-amyloid-fibrils-formed-by-short-amyloidogenic-peptides
#19
REVIEW
Zahraa S Al-Garawi, Kyle L Morris, Karen E Marshall, Jutta Eichler, Louise C Serpell
Amyloidogenic peptides are well known for their involvement in diseases such as type 2 diabetes and Alzheimer's disease. However, more recently, amyloid fibrils have been shown to provide scaffolding and protection as functional materials in a range of organisms from bacteria to humans. These roles highlight the incredible tensile strength of the cross-β amyloid architecture. Many amino acid sequences are able to self-assemble to form amyloid with a cross-β core. Here we describe our recent advances in understanding how sequence contributes to amyloidogenicity and structure...
December 6, 2017: Interface Focus
https://www.readbyqxmd.com/read/29142197/proteotoxicity-in-cardiac-amyloidosis-amyloidogenic-light-chains-affect-the-levels-of-intracellular-proteins-in-human-heart-cells
#20
Esther Imperlini, Massimiliano Gnecchi, Paola Rognoni, Eduard Sabidò, Maria Chiara Ciuffreda, Giovanni Palladini, Guadalupe Espadas, Francesco Mattia Mancuso, Margherita Bozzola, Giuseppe Malpasso, Veronica Valentini, Giuseppina Palladini, Stefania Orrù, Giovanni Ferraro, Paolo Milani, Stefano Perlini, Francesco Salvatore, Giampaolo Merlini, Francesca Lavatelli
AL amyloidosis is characterized by widespread deposition of immunoglobulin light chains (LCs) as amyloid fibrils. Cardiac involvement is frequent and leads to life-threatening cardiomyopathy. Besides the tissue alteration caused by fibrils, clinical and experimental evidence indicates that cardiac damage is also caused by proteotoxicity of prefibrillar amyloidogenic species. As in other amyloidoses, the damage mechanisms at cellular level are complex and largely undefined. We have characterized the molecular changes in primary human cardiac fibroblasts (hCFs) exposed in vitro to soluble amyloidogenic cardiotoxic LCs from AL cardiomyopathy patients...
November 15, 2017: Scientific Reports
keyword
keyword
77366
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"