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https://www.readbyqxmd.com/read/28226286/peripheral-nerve-ultrasonography-in-patients-with-transthyretin-amyloidosis
#1
Simon Podnar, Stayko Sarafov, Ivailo Tournev, Gregor Omejec, Janez Zidar
OBJECTIVE: To systematically study peripheral nerve morphology in patients with transthyretin (TTR) amyloidosis and TTR gene mutation carriers using high-resolution ultrasonography (US). METHODS: In this prospective cross-sectional study we took a structured history, performed neurological examination, and measured peripheral nerve cross-sectional areas (CSAs) bilaterally at 28 standard locations using US. Demographic and US findings were compared to controls. RESULTS: Peripheral nerve CSAs were significantly larger in 33 patients with familial amyloid polyneuropathy (FAP) compared to 50 controls, most dramatically at the common entrapment sites (median nerve at the wrist, ulnar nerve at the elbow), and in the proximal nerve segments (median nerve in the upper arm, sciatic nerve in the thigh)...
January 29, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28220836/nucleobindin-1-binds-to-multiple-types-of-pre-fibrillar-amyloid-and-inhibits-fibrillization
#2
Alessandra Bonito-Oliva, Shahar Barbash, Thomas P Sakmar, W Vallen Graham
During amyloid fibril formation, amyloidogenic polypeptides misfold and self assemble into soluble pre-fibrillar aggregates, i.e., protofibrils, which elongate and mature into insoluble fibrillar aggregates. An emerging class of chaperones, chaperone-like amyloid binding proteins (CLABPs), has been shown to interfere with aggregation of particular misfolded amyloid peptides or proteins. We have discovered that the calcium-binding protein nuclebindin-1 (NUCB1) is a novel CLABP. We show that NUCB1 inhibits aggregation of islet-amyloid polypeptide associated with type 2 diabetes mellitus, a-synuclein associated with Parkinson's disease, transthyretin V30M mutant associated with familial amyloid polyneuropathy, and Aβ42 associated with Alzheimer's disease by stabilizing their respective protofibril intermediates...
February 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28218749/mechanistic-basis-for-the-recognition-of-a-misfolded-protein-by-the-molecular-chaperone-hsp90
#3
Javier Oroz, Jin Hae Kim, Bliss J Chang, Markus Zweckstetter
The critical toxic species in over 40 human diseases are misfolded proteins. Their interaction with molecular chaperones such as Hsp90, which preferentially interacts with metastable proteins, is essential for the blocking of disease progression. Here we used nuclear magnetic resonance (NMR) spectroscopy to determine the three-dimensional structure of the misfolded cytotoxic monomer of the amyloidogenic human protein transthyretin, which is characterized by the release of the C-terminal β-strand and perturbations of the A-B loop...
February 20, 2017: Nature Structural & Molecular Biology
https://www.readbyqxmd.com/read/28217795/understanding-the-microscopic-binding-mechanism-of-hydroxylated-and-sulfated-polybrominated-diphenyl-ethers-with-transthyretin-by-molecular-docking-molecular-dynamics-simulations-and-binding-free-energy-calculations
#4
Huiming Cao, Yuzhen Sun, Ling Wang, Chunyan Zhao, Jianjie Fu, Aiqian Zhang
Polybrominated diphenyl ethers (PBDEs), one typical type of persistent environmental contaminant, have toxicological effects such as disrupting thyroid homeostasis in the human body. The high binding affinities of hydroxylated metabolites of PBDEs (OH-PBDEs) with transthyretin (TTR) were considered to be one major reason for their extraordinary capacity of passing through the blood-brain barrier via competitive thyroid hormone (T4) transport protein binding. Recent findings showed that sulfated PBDEs can be formed in human liver cytosol as phase-II metabolites...
February 20, 2017: Molecular BioSystems
https://www.readbyqxmd.com/read/28217771/detection-inhibition-and-disintegration-of-amyloid-fibrils-the-role-of-optical-probes-and-macrocyclic-receptors
#5
Achikanath C Bhasikuttan, Jyotirmayee Mohanty
Amyloid fibrils are formed by the aberrant aggregation of proteins into highly ordered β-sheet structures and are believed to be the root cause of several neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, Prion diseases, etc. and have been the subject of extensive biochemical, biophysical and clinical studies. Developing methods for the early detection of fibril formation using optical spectroscopic techniques and inhibition/disintegration of amyloid fibrils/plaques by introducing small molecules have been a major challenge to establish a clinically facile therapeutic intervention to combat these neurodegenerative diseases...
February 20, 2017: Chemical Communications: Chem Comm
https://www.readbyqxmd.com/read/28217248/iatrogenic-amyloid-polyneuropathy-after-domino-liver-transplantation
#6
REVIEW
Diana Mnatsakanova, Saša A Živković
Liver transplantation has been used in treatment of transthyretin amyloidosis, and some patients undergo domino liver transplantation (DLT) with explanted liver being transplanted to another patient with liver failure as the liver is otherwise usually functionally normal. Until end of 2015, there were 1154 DLT performed worldwide. DLT for transthyretin amyloidosis is associated with the risk of developing de novo systemic amyloidosis and amyloid neuropathy, and the risk may be greater with some non-Val30Met mutations...
January 28, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28213611/transthyretin-amyloidosis-an-under-recognized-neuropathy-and-cardiomyopathy
#7
REVIEW
Natalie J Galant, Per Westermark, Jeffrey N Higaki, Avijit Chakrabartty
Transthyretin (TTR) amyloidosis (ATTR amyloidosis) is an underdiagnosed and important type of cardiomyopathy and/or polyneuropathy that requires increased awareness within the medical community. Raising awareness among clinicians about this type of neuropathy and lethal form of heart disease is critical for improving earlier diagnosis and the identification of patients for treatment. The following review summarizes current criteria used to diagnose both hereditary and wild-type ATTR (ATTRwt) amyloidosis, tools available to clinicians to improve diagnostic accuracy, available and newly developing therapeutics, as well as a brief biochemical and biophysical background of TTR amyloidogenesis...
March 1, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28202889/treatment-of-transthyretin-familial-amyloid-polyneuropathy-with-tafamidis-a-case-report
#8
Yoshimichi Miyazaki
INTRODUCTION: Familial amyloid polyneuropathy (FAP) is a rare hereditary disorder caused by mutations in the transthyretin (TTR) gene. Tafamidis is a TTR stabilizer able to prevent TTR tetramer dissociation, and several studies have demonstrated its safety and efficacy at slowing the progression of neuropathy in FAP caused by the TTR Val30Met mutation. However, nerve conduction study (NCS) and electromyography (EMG) results have yet to be reported in relation to FAP progression during tafamidis therapy...
2017: Nihon Ronen Igakkai Zasshi. Japanese Journal of Geriatrics
https://www.readbyqxmd.com/read/28196416/blood-proteomic-profiling-in-inherited-attrm-and-acquired-attrwt-forms-of-transthyretin-associated-cardiac-amyloidosis
#9
Gloria G Chan, Clarissa M Koch, Lawreen H Connors
Transthyretin-associated forms of cardiac amyloidosis are fatal protein misfolding diseases that can be inherited (ATTRm) or acquired (ATTRwt). An accurate diagnosis of ATTR amyloidosis can be challenging as biopsy evidence, usually from the affected organ, is required. Precise biomarkers for ATTR disease identification and monitoring are undiscovered, disease-specific therapeutic options are needed, and the current understanding of ATTR molecular pathogenesis is limited. The aim of this study was to investigate and compare the serum proteomes in ATTRm and ATTRwt cardiac amyloidosis to identify differentially expressed blood proteins that were disease-specific...
February 23, 2017: Journal of Proteome Research
https://www.readbyqxmd.com/read/28196196/identification-of-transthyretin-cardiac-amyloidosis-using-serum-retinol-binding-protein-4-and-a-clinical-prediction-model
#10
Marios Arvanitis, Clarissa M Koch, Gloria G Chan, Celia Torres-Arancivia, Michael P LaValley, Daniel R Jacobson, John L Berk, Lawreen H Connors, Frederick L Ruberg
Importance: Transthyretin cardiac amyloidosis (ATTR) is an underrecognized cause of heart failure (HF) in older individuals, owing in part to difficulty in diagnosis. ATTR can result from substitution of valine for isoleucine at codon 122 of the transthyretin (TTR) gene (V122I), present in 3.43% of African American individuals. Objective: To examine whether serum retinol-binding protein 4 (RBP4), an endogenous TTR ligand, could be used as a diagnostic test for ATTR V122I amyloidosis...
February 8, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28176254/-18-fluorine-sodium-fluoride-positron-emission-tomography-a-potential-biomarker-of-transthyretin-cardiac-amyloidosis
#11
Rachelle Morgenstern, Randy Yeh, Adam Castano, Mathew S Maurer, Sabahat Bokhari
BACKGROUND: Non-invasive imaging to diagnose and quantify amyloid load, progression, and response to treatment are central for the care of patients with cardiac amyloidosis. (18)Fluorine-labeled sodium fluoride ((18)F-NaF) is a widely available radioisotope and PET imaging allows for absolute quantification of tracer uptake. METHODS: Patients with biopsy-proven transthyretin (ATTR-CA) and light-chain cardiac amyloidosis (AL) (3 ATTRwt, 2 ATTRV122I, 2 AL) and controls (n = 5), underwent (18)F-NaF PET imaging...
February 7, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28163385/an-oxidized-reduced-state-of-plasma-albumin-reflects-malnutrition-due-to-an-insufficient-diet-in-rats
#12
Masashi Kuwahata, Mari Hasegawa, Yukiko Kobayashi, Yasuaki Wada, Yasuhiro Kido
We examined whether protein- and food-intake restrictions modulate the oxidized/reduced state of plasma albumin in Sprague-Dawley rats. Rats were fed a 3%, 5%, 10% or 20% casein diet for 2 weeks. The plasma albumin concentration significantly decreased with decreasing protein intake. However, no significant difference in plasma albumin concentration was seen between rats fed the 5% or 10% casein diet. In rats fed the 5% casein diet, the percentage of mercaptalbumin within total plasma albumin was significantly lower and that of nonmercaptalbumin-1 was significantly higher than in rats fed the 10% casein diet...
January 2017: Journal of Clinical Biochemistry and Nutrition
https://www.readbyqxmd.com/read/28163147/senile-cardiac-amyloidosis-clinical-manifestations-and-non-invasive-diagnostic-approach
#13
Dimitrios Varvarousis, Kali Polytarchou, Nikolaos Daskalopoulos, Ioannis Mantas
Cardiac amyloidosis (CA) refers to an uncommon storage disease leading to restrictive cardiomyopathy and conduction abnormalities. Survival is generally poor but depends strongly on the amyloid type. Wild type transthyretin amyloidosis or senile cardiac amyloidosis (SCA) is a disease of the >60-year age group, has a relatively better prognosis among the various amyloid types and is considered to be underdiagnosed. We describe a case of a patient with clinical signs and symptoms of restrictive cardiomyopathy and a suggested diagnosis of SCA and discuss the diagnostic and therapeutic approach...
February 2, 2017: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
https://www.readbyqxmd.com/read/28159995/prognostic-utility-of-the-perugini-grading-of-99mtc-dpd-scintigraphy-in-transthyretin-attr-amyloidosis-and-its-relationship-with-skeletal-muscle-and-soft-tissue-amyloid
#14
David F Hutt, Marianna Fontana, Maria Burniston, Ann-Marie Quigley, Aviva Petrie, James C Ross, Joanne Page, Ana Martinez-Naharro, Ashutosh D Wechalekar, Helen J Lachmann, Candida C Quarta, Tamer Rezk, Shameem Mahmood, Sajitha Sachchithanantham, Taryn Youngstein, Carol J Whelan, Thirusha Lane, Janet A Gilbertson, Dorota Rowczenio, Philip N Hawkins, Julian D Gillmore
No abstract text is available yet for this article.
February 4, 2017: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28155044/transthyretin-cardiac-amyloid-and-aortic-stenosis-in-the-elderly-the-role-of-nuclear-imaging
#15
Elisa Merli, Eleonora Del Giudice, Athanassios Antonopoulos, Gianadrea Amadei, Elisabetta Varani
No abstract text is available yet for this article.
February 2, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28152524/tafamidis-for-the-treatment-of-hereditary-transthyretin-amyloid-cardiomyopathy-a-case-report
#16
Teppei Fujita, Takayuki Inomata, Toyoji Kaida, Yuichirou Iida, Yuki Ikeda, Takeru Nabeta, Shunsuke Ishii, Emi Maekawa, Takashi Naruke, Toshimi Koitabashi, Eiji Kitamura, Yoshiki Sekijima, Junya Ako
Tafamidis meglumine is a novel medicine that has been shown to slow the progression of peripheral neurological impairment in patients with hereditary transthyretin amyloidosis (ATTR). However, the efficacy of tafamidis against ATTR-related cardiac amyloidosis remains unclear. A 72-year-old woman had cardiac hypertrophy and axonopathy in her lower legs. Endomyocardial biopsy revealed an infiltrative cardiomyopathy consistent with amyloidosis. Immunostaining and genetic studies confirmed the diagnosis of ATTR, and tafamidis was started subsequently...
February 3, 2017: Cardiology
https://www.readbyqxmd.com/read/28150156/technetium-99m-pyrophosphate-radioisotope-for-diagnosis-and-prognosis-of-transthyretin-cardiac-amyloidosis-a-call-for-collaboration
#17
EDITORIAL
Adam Castaño, Mathew S Maurer, Sabahat Bokhari
No abstract text is available yet for this article.
February 1, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28145509/serum-protein-changes-in-a-rat-model-of-chronic-pain-show-a-correlation-between-animal-and-humans
#18
Elisa Bellei, Antonietta Vilella, Emanuela Monari, Stefania Bergamini, Aldo Tomasi, Aurora Cuoghi, Simona Guerzoni, Letizia Manca, Michele Zoli, Luigi Alberto Pini
In previous works we showed the overexpression of some proteins in biological fluids from patients suffering chronic pain. In this proteomic study we analysed serum from a rat model of neuropathic pain obtained by the chronic constriction injury (CCI) of sciatic nerve, at two time intervals, 2 and 5 weeks after the insult, to find proteins involved in the expression or mediation of pain. Sham-operated and CCI rats were treated with saline or indomethacin. Two weeks after ligation, we identified three serum proteins overexpressed in CCI rats, two of which, alpha-1-macroglobulin and vitamin D-binding protein (VDBP), remained increased 5 weeks post-surgery; at this time interval, we found increased levels of further proteins, namely apolipoprotein A-I (APOA1), apolipoprotein E (APOE), prostaglandin-H2 D-isomerase (PTGDS) and transthyretin (TTR), that overlap the overexpressed proteins found in humans...
February 1, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28143668/resting-energy-expenditure-in-the-risk-assessment-of-anticancer-treatments
#19
Anne Jouinot, Clara Vazeille, Jean Philippe Durand, Olivier Huillard, Pascaline Boudou-Rouquette, Romain Coriat, Jeanne Chapron, Nathalie Neveux, Jean Pascal De Bandt, Jerome Alexandre, Luc Cynober, Francois Goldwasser
BACKGROUND & AIMS: Alterations of nutritional and performance status (PS) are associated with higher risk of chemotherapy toxicity. Increased resting energy expenditure (REE) is frequent in cancer patients and may contribute to cachexia. We investigated whether abnormal energetic metabolism could predict early acute limiting toxicities (ELT) of anticancer treatments. METHODS: In this observational monocentric study, REE was measured by indirect calorimetry before treatment initiation...
January 19, 2017: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28134916/rescue-of-retinal-morphology-and-function-in-a-humanized-mouse-at-the-mouse-retinol-binding-protein-locus
#20
Li Liu, Tomohiro Suzuki, Jingling Shen, Shigeharu Wakana, Kimi Araki, Ken-Ichi Yamamura, Lei Lei, Zhenghua Li
Retinol-binding protein RBP4 is the specific carrier for retinol in the blood. We previously produced a Rbp4-deficient (Rbp4(-/-)) mouse that showed electroretinogram (ERG) abnormalities, accompanied by histological and electron-microscopic changes such as fewer synapses in the inner plexiform layer in the central retina. To address whether human RBP4 gene expression can rescue the phenotypes observed in Rbp4(-/-) mice, we produced a humanized (Rbp4(hRBP4orf/ hRBP4orf)) mouse with a human RBP4 open reading frame in the mouse Rbp4 locus using a Cre-mutant lox recombination system...
January 30, 2017: Laboratory Investigation; a Journal of Technical Methods and Pathology
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