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Transthyretin

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https://www.readbyqxmd.com/read/28333852/re-intervention-in-de-novo-vitreous-opacities-after-pars-plana-vitrectomy-in-familial-amyloidotic-polyneuropathy-ttr-val30metportuguese-patients
#1
Natália Novais Ferreira, David Afonso Cunha Dias, Rui Pedro Afonso Carvalho, Maria Teresa Pardal Monteiro Coelho
PURPOSE: To report management of de novo vitreous amyloid opacities after previous pars plana vitrectomy in familial amyloidotic polyneuropathy transthyretin Val30Met. METHODS: This work is a retrospective observational consecutive case series of five eyes of four patients. Demographic data, transthyretin mutation involved, age at the beginning of disease, duration of disease, treatment (liver transplant or tafamidis), time between vitrectomy and re-intervention, and ophthalmologic changes were evaluated...
March 22, 2017: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/28329248/clinical-characteristics-of-wild-type-transthyretin-cardiac-amyloidosis-disproving-myths
#2
Esther González-López, Christian Gagliardi, Fernando Dominguez, Cristina Candida Quarta, F Javier de Haro-Del Moral, Agnese Milandri, Clara Salas, Mario Cinelli, Marta Cobo-Marcos, Massimiliano Lorenzini, Enrique Lara-Pezzi, Serena Foffi, Luis Alonso-Pulpon, Claudio Rapezzi, Pablo Garcia-Pavia
Aims: Wild-type transthyretin amyloidosis (ATTRwt) is mostly considered a disease predominantly of elderly male, characterized by concentric LV hypertrophy, preserved LVEF, and low QRS voltages. We sought to describe the characteristics of a large cohort of ATTRwt patients to better define the disease. Methods and results: Clinical findings of consecutive ATTRwt patients diagnosed at 2 centres were reviewed. ATTRwt was diagnosed histologically or non-invasively (LV hypertrophy ≥12 mm, intense cardiac uptake at 99mTc-DPD scintigraphy and AL exclusion)...
March 1, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28326465/pet-ct-evaluation-of-amyloid-systemic-involvement-with-18-f-florbetaben-in-patient-with-proved-cardiac-amyloidosis-a-case-report
#3
Dario Genovesi, Giuseppe Vergaro, Michele Emdin, Assuero Giorgetti, Paolo Marzullo
BACKGROUND: One of the most frequent disorders which lead to cardiac amyloidosis is transthyretin-related amyloidosis (ATTR). Some PET radio-pharmaceuticals for the detection of beta-amyloid deposits within the brain have shown to be able to detect also cardiac amyloid deposits. We present a case of a man with ATTR studied with [(18)F]-florbetaben PET-CT. RESULTS: Total-body scan showed a moderate uptake in the bone marrow, especially in correspondence of the vertebral column, while no significant myocardial uptake was present...
March 21, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28314625/transthyretin-concentrations-in-acute-stroke-patients-predict-convalescent-rehabilitation
#4
Naofumi Isono, Yuki Imamura, Keiko Ohmura, Norihide Ueda, Shinji Kawabata, Motomasa Furuse, Toshihiko Kuroiwa
OBJECTIVE: For stroke patients, intensive nutritional management is an important and effective component of inpatient rehabilitation. Accordingly, acute care hospitals must detect and prevent malnutrition at an early stage. Blood transthyretin levels are widely used as a nutritional monitoring index in critically ill patients. Here, we had analyzed the relationship between the transthyretin levels during the acute phase and Functional Independence Measure in stroke patients undergoing convalescent rehabilitation...
March 14, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28301312/identification-of-two-meloidogyne-hapla-genes-and-an-investigation-of-their-roles-in-the-plant-nematode-interaction
#5
Cynthia Gleason, Frederik Polzin, Samer S Habash, Lei Zhang, Jan Utermark, Florian M W Grundler, Abdelnaser Elashry
Root-knot nematodes are soil-borne pathogens that invade and establish feeding sites in plant roots. They have an extremely broad host range, including most vascular plants. During infection of a susceptible host, root-knot nematodes secrete molecules called effectors that help them establish an intimate interaction with the plant and, at the same time, allow them to evade or suppress plant immune responses. Despite the fact that Meloidogyne hapla is a significant pest on several food crops, no effectors have been characterized from this root-knot nematode species thus far...
February 2017: Molecular Plant-microbe Interactions: MPMI
https://www.readbyqxmd.com/read/28298647/inhibition-of-the-mechano-enzymatic-amyloidogenesis-of-transthyretin-role-of-ligand-affinity-binding-cooperativity-and-occupancy-of-the-inner-channel
#6
Guglielmo Verona, P Patrizia Mangione, Sara Raimondi, Sofia Giorgetti, Giulia Faravelli, Riccardo Porcari, Alessandra Corazza, Julian D Gillmore, Philip N Hawkins, Mark B Pepys, Graham W Taylor, Vittorio Bellotti
Dissociation of the native transthyretin (TTR) tetramer is widely accepted as the critical step in TTR amyloid fibrillogenesis. It is modelled by exposure of the protein to non-physiological low pH in vitro and is inhibited by small molecule compounds, such as the drug tafamidis. We have recently identified a new mechano-enzymatic pathway of TTR fibrillogenesis in vitro, catalysed by selective proteolytic cleavage, which produces a high yield of genuine amyloid fibrils. This pathway is efficiently inhibited only by ligands that occupy both binding sites in TTR...
March 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28295152/causally-treatable-hereditary-neuropathies-in-fabry-s-disease-transthyretin-related-familial-amyloidosis-and-pompe-s-disease
#7
REVIEW
J Finsterer, J Wanschitz, S Quasthoff, S Iglseder, W Löscher, W Grisold
OBJECTIVES: Most acquired neuropathies are treatable, whereas genetic neuropathies respond to treatment in Fabry's disease (FD), transthyretin-related familial amyloidosis (TTR-FA), and Pompe's disease (PD). This review summarizes and discusses recent findings and future perspectives concerning etiology, pathophysiology, clinical presentation, diagnosis, treatment, and outcome of neuropathy in FD, TTR-FA, and PD. METHODS: Literature review. RESULTS: Neuropathy in FD concerns particularly small, unmyelinated, or myelinated sensory fibers (small fiber neuropathy [SFN]) and autonomic fibers, manifesting as acroparesthesias, Fabry's crises, or autonomous disturbances...
March 12, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/28281017/heart-transplantation-in-cardiac-amyloidosis
#8
REVIEW
Matthew Sousa, Gregory Monohan, Navin Rajagopalan, Alla Grigorian, Maya Guglin
"Cardiac amyloidosis" is the term commonly used to reflect the deposition of abnormal protein amyloid in the heart. This process can result from several different forms, most commonly from light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis, which in turn can represent wild-type (ATTRwt) or genetic form. Regardless of the origin, cardiac involvement is usually associated with poor prognosis, especially in AL amyloidosis. Although several treatment options, including chemotherapy, exist for different forms of the disease, cardiac transplantation is increasingly considered...
March 9, 2017: Heart Failure Reviews
https://www.readbyqxmd.com/read/28272196/transthyretin-related-hereditary-amyloidosis-with-recurrent-vomiting-and-renal-insufficiency-as-the-initial-presentation-a-case-report
#9
Jing Xu, Meng Yang, Xiaoxia Pan, Xialian Yu, Jingyuan Xie, Hong Ren, Xiao Li, Nan Chen
RATIONALE: Hereditary amyloidosis is diagnosed worldwidely with an increasing incidence. As the most common form, transthyretin-related hereditary amyloidosis (ATTR amyloidosis) is an autosomal dominant inherited disease due to mutations of TTR. Over the past several decades, more than 130 mutations have been reported. Previous studies suggested that ATTR amyloidosis initially showed polyneuropathy and autonomic dysfunction but later involving many visceral organs, such as kidney. PATIENT CONCERNS: A young proband carrying TTR p...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28271559/novel-screening-for-transthyretin-amyloidosis-by-using-fat-ultrasonography
#10
Yohei Misumi, Mitsuharu Ueda, Taro Yamashita, Teruaki Masuda, Yumiko Kinoshita, Masayoshi Tasaki, Terumasa Nagase, Yukio Ando
We aimed to assess the possibility of using a noninvasive screening method for hereditary transthyretin amyloidosis by means of abdominal fat ultrasonography. Quantitative analysis of ultrasound B-mode images demonstrated a significant increase in mean echogenicity and a loss of the normal structure of the layers of fat tissue in patients with hereditary transthyretin amyloidosis (n = 19). The ultrasound features of the fat tissue and the degree of amyloid deposition seen histopathologically showed a significant correlation...
March 8, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28257828/directional-thyroid-hormone-distribution-via-the-blood-stream-to-target-sites
#11
Susanne T Janssen, Onno E Janssen
Thyroid hormones are bound to three major serum transport proteins, thyroxin-binding globulin (TBG), transthyretin (TTR) and human serum albumin (HSA). TBG has the strongest affinity for thyroid hormones, TTR is also found in the cerebrospinal fluid and HSA is the most abundant protein in plasma. Combination defects of either a high affinity TTR or HSA variant do not compensate TBG deficiency, underscoring the dominant role of TBG among the thyroid hormone transport proteins. On the other hand, coexistence of raised affinity TTR and HSA variants causes an augmented hyperthyroxinemia...
February 28, 2017: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/28257095/micronutrient-status-in-153-patients-with-anorexia-nervosa
#12
Najate Achamrah, Moïse Coëffier, Agnès Rimbert, Jocelyne Charles, Vanessa Folope, André Petit, Pierre Déchelotte, Sébastien Grigioni
Micronutrient status in Anorexia Nervosa (AN) has been poorly documented and previous data are often contradictory. We aimed to assess micronutrient status in a large population of AN patients. The relationships between micronutrient status and body composition were also determined. Anthropometric, biochemical parameters and body composition data were collected at referral in 153 patients with AN (28.5 ± 11 years). At least one trace element deficit was observed in almost half of patients; the most frequent was selenium deficit (40% of patients)...
March 2, 2017: Nutrients
https://www.readbyqxmd.com/read/28256403/the-utility-of-99m-tc-dpd-scintigraphy-in-the-diagnosis-of-cardiac-amyloidosis-an-australian-experience
#13
Peter T Moore, Matthew K Burrage, Emily Mackenzie, W Philip Law, Dariusz Korczyk, Peter Mollee
BACKGROUND: The uptake of bone-seeking radiotracers in the amyloid heart is well recognised. 99(m)Tc-DPD has been shown to be highly sensitive for cardiac transthyretin (ATTR) amyloid in an overseas population, but is not registered for use in Australia. We explored its utility as a diagnostic tool within our population. METHODS: Patients diagnosed with AL and ATTR (wild-type and inherited) cardiac amyloidosis were prospectively recruited from the Princess Alexandra Hospital Amyloidosis Centre...
February 16, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28249735/evolution-of-thyroid-hormone-distributor-proteins
#14
Thomas R McLean, Michelle M Rank, Peter M Smooker, Samantha J Richardson
Thyroid hormones (THs) are evolutionarily old hormones, having effects on metabolism in bacteria, invertebrates and vertebrates. THs bind specific distributor proteins (THDPs) to ensure their efficient distribution through the blood and cerebrospinal fluid in vertebrates. Albumin is a THDP in the blood of all studied species of vertebrates, so may be the original vertebrate THDP. However, albumin has weak affinity for THs. Transthyretin (TTR) has been identified in the blood across different lineages in adults vs juveniles...
February 27, 2017: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/28249176/identification-of-potential-serum-biomarkers-in-pigs-at-early-stage-after-lipopolysaccharide-injection
#15
Chao Yin, Weifeng Liu, Zhiqing Liu, Yanping Huang, Le Ci, Ruqian Zhao, Xiaojing Yang
The identification of useful biological indicators to monitor the body response before the presentation of clinical diseases has practical value in livestock production. To identify potential biomarkers in pigs at the early stage during inflammation, 12 pigs were intramuscularly injected with 2mL of Lipopolysaccharide (LPS, 15μg/kg BW) or saline. Serum protein expression profiles were detected with two-dimensional electrophoresis (2-DE) and matrix-assisted laser desorption ionization time of flight mass spectrometry (MALDI-TOF-MS) techniques...
February 17, 2017: Research in Veterinary Science
https://www.readbyqxmd.com/read/28242306/characteristics-of-the-brown-hagfish-paramyxine-atami-transthyretin-metal-ion-dependent-thyroid-hormone-binding
#16
Shunsuke Suzuki, Kentaro Kasai, Norihito Nishiyama, Akinori Ishihara, Kiyoshi Yamauchi
Transthyretin (TTR) is a vertebrate-specific protein involved in thyroid hormone distribution in plasma, and its gene is thought to have emerged by gene duplication from the gene for the ancient TTR-related protein, 5-hydroxyisourate hydrolase, at some early stage of chordate evolution. We investigated the molecular and hormone-binding properties of the brown hagfish Paramyxine atami TTR. The amino acid sequence deduced from the cloned hagfish TTR cDNA shared 33-50% identities with those of other vertebrate TTRs but less than 24% identities with those of vertebrate and deuterostome invertebrate 5-hydroxyisourate hydrolases...
February 24, 2017: General and Comparative Endocrinology
https://www.readbyqxmd.com/read/28226286/peripheral-nerve-ultrasonography-in-patients-with-transthyretin-amyloidosis
#17
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)...
April 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
#18
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
#19
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
#20
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
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