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Transthyretin

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https://www.readbyqxmd.com/read/28647518/increasing-the-accuracy-of-proteomic-typing-by-decellularisation-of-amyloid-tissue-biopsies
#1
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/28647184/identification-of-novel-biomarkers-for-adult-onset-immunodeficiency-aoid-syndrome-using-serum-proteomics
#2
Jeerang Wongtrakul, Thananya Thongtan, Sittiruk Roytrakul, Jutarat Praparattanapan, Jiraprapa Wipasa, Benjawan Kumrapich, Khuanchai Supparatpinyo
OBJECTIVE: To identify the candidate protein biomarkers of adult-onset-immunodeficiency (AOID) syndrome using serum proteomics. METHODS: Screening and verification phases were performed in the study. A total of 97 serum samples were classified into three groups: AOID patients with opportunistic infections (active AOID), AOID patients without opportunistic infections (inactive AOID), and healthy control. In the screening phase, pooled sera collected from patients and healthy control in each group were separated by 2D-gel electrophoresis, analyzed for differentially expressed proteins and identified for biomarkers using LC/MS...
May 2017: Asian Pacific Journal of Tropical Medicine
https://www.readbyqxmd.com/read/28646538/a-novel-attr-l32v-mutation-causes-familial-amyloid-polyneuropathy-in-a-bolivian-family
#3
P L Martínez-Ulloa, M Vallejo, I Corral, N García-Barragán, A Alcazar, Emma Martínez-Alonso, J Martínez-Poles, H Pian, A Jiménez-Escrig
We report a new transthyretin (ATTR) gene c.272C>G mutation and variant protein, p.Leu32Val, in a kindred of Bolivian origin with a rapid progressive peripheral neuropathy and cardiomyopathy. Three individuals from a kindred with peripheral nerve and cardiac amyloidosis were examined. Analysis of the TTR gene was performed by Sanger direct sequencing. Neuropathologic examination was obtained on the index patient with mass spectrometry study of the ATTR deposition. Direct DNA sequence analysis of exons 2, 3 and 4 of the TTR gene demonstrated a c...
June 24, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28646497/geometrical-principles-of-homomeric-%C3%AE-barrels-and-%C3%AE-helices-application-to-modelling-amyloid-protofilaments
#4
Steven Hayward, E James Milner-White
Examples of homomeric β-helices and β-barrels have recently emerged. Here we generalise the theory for the shear number in β-barrels to encompass β-helices and homomeric structures. We introduce the concept of the "β-strip", the set of parallel or antiparallel neighbouring strands, from which the whole helix can be generated giving it n-fold rotational symmetry. In this context the shear number is interpreted as the sum around the helix of the fixed register shift between neighbouring identical β-strips...
June 24, 2017: Proteins
https://www.readbyqxmd.com/read/28637305/left-atrial-structure-and-function-in-cardiac-amyloidosis
#5
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/28636639/predictive-value-of-serum-transthyretin-for-outcome-in-acute-ischemic-stroke
#6
Wojciech Ambrosius, Slawomir Michalak, Radosław Kazmierski, Natalia Andrzejewska, Wojciech Kozubski
INTRODUCTION: The impact of choroid plexus with its blood-cerebrospinal fluid barrier in the ischemic stroke pathology is poorly explored. Transthyretin (TTR) is a protein synthesized in liver and just in choroid plexus. OBJECTIVES: The current study was designed to assess the prognostic value of serum TTR for functional outcome (at the time of hospital discharge) and long-term (one-year) overall mortality in ischemic stroke patients. PATIENTS AND METHODS: We conducted a prospective observational study...
2017: PloS One
https://www.readbyqxmd.com/read/28635419/multimodal-imaging-gd-nanoparticles-functionalized-with-pittsburgh-compound-b-or-a-nanobody-for-amyloid-plaques-targeting
#7
Jonathan Pansieri, Marie Plissonneau, Nathalie Stransky-Heilkron, Mireille Dumoulin, Laurence Heinrich-Balard, Pascaline Rivory, Jean-François Morfin, Eva Toth, Maria Joao Saraiva, Eric Allémann, Olivier Tillement, Vincent Forge, François Lux, Christel Marquette
AIM: Gadolinium-based nanoparticles were functionalized with either the Pittsburgh compound B or a nanobody (B10AP) in order to create multimodal tools for an early diagnosis of amyloidoses. MATERIALS & METHODS: The ability of the functionalized nanoparticles to target amyloid fibrils made of β-amyloid peptide, amylin or Val30Met-mutated transthyretin formed in vitro or from pathological tissues was investigated by a range of spectroscopic and biophysics techniques including fluorescence microscopy...
June 21, 2017: Nanomedicine
https://www.readbyqxmd.com/read/28634083/effects-of-the-soya-isoflavone-genistein-in-early-life-stages-of-the-senegalese-sole-solea-senegalensis-thyroid-estrogenic-and-metabolic-biomarkers
#8
Carmen Sarasquete, Maria Úbeda-Manzanaro, Juan Bosco Ortiz-Delgado
This study examines the effects induced by environmentally relevant concentrations of the isoflavone genistein (3 mg/L and 10 mg/L) during early life stages of the Senegalese sole. Throughout the hypothalamus-pituitary-thyroid (HPT) axis, several neurohormonal regulatory thyroid signalling patterns (thyroglobulin/Tg, thyroid peroxidase/TPO, transthyretin/TTR, thyroid receptors/TRβ, and iodothrynonine deiodinases, Dio2 and Dio3) were analysed. Furthermore, the expression patterns of estrogen receptor ERβ and haemoprotein Cyp1a were also evaluated...
June 17, 2017: General and Comparative Endocrinology
https://www.readbyqxmd.com/read/28627187/-is-plasma-selenium-correlated-to-transthyretin-levels-in-critically-ill-patients
#9
Renata G B O N Freitas, Roberto Jose Negrão Nogueira, Silvia Maria Franciscato Cozzolino, Ana Carolina Junqueira Vasques, Matthew Thomas Ferreira, Gabriel Hessel
BACKGROUND: Selenium is an essential trace element, but critically ill patients using total parenteral nutrition (PN) do not receive selenium because this mineral is not commonly offered. Threfore, the eval uation of plasma selenium levels is very important for treating or preventing this deficiency. Recent studies have shown that transthyretin may reflect the selenium intake and could be considered a biomarker. However, this issue is still little explored in the literature. OBJECTIVE: This study aims to investigate the correlation of transthyretin with the plasma selenium of critically ill patients receiving PN...
June 5, 2017: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/28625364/semi-quantitative-models-for-identifying-potent-and-selective-transthyretin-amyloidogenesis-inhibitors
#10
Stephen Connelly, David E Mortenson, Sungwook Choi, Ian A Wilson, Evan T Powers, Jeffery W Kelly, Steven M Johnson
Rate-limiting dissociation of the tetrameric protein transthyretin (TTR), followed by monomer misfolding and misassembly, appears to cause degenerative diseases in humans known as the transthyretin amyloidoses, based on human genetic, biochemical and pharmacologic evidence. Small molecules that bind to the generally unoccupied thyroxine binding pockets in the native TTR tetramer kinetically stabilize the tetramer, slowing subunit dissociation proportional to the extent that the molecules stabilize the native state over the dissociative transition state-thereby inhibiting amyloidogenesis...
May 26, 2017: Bioorganic & Medicinal Chemistry Letters
https://www.readbyqxmd.com/read/28624194/sustained-inhibition-of-hbv-replication-in%C3%A2-vivo-after-systemic-injection-of-aavs-encoding-artificial-antiviral-primary-micrornas
#11
Mohube Betty Maepa, Abdullah Ely, Wayne Grayson, Patrick Arbuthnot
Chronic infection with hepatitis B virus (HBV) remains a problem of global significance and improving available treatment is important to prevent life-threatening complications arising in persistently infected individuals. HBV is susceptible to silencing by exogenous artificial intermediates of the RNA interference (RNAi) pathway. However, toxicity of Pol III cassettes and short duration of silencing by effectors of the RNAi pathway may limit anti-HBV therapeutic utility. To advance RNAi-based HBV gene silencing, mono- and trimeric artificial primary microRNAs (pri-miRs) derived from pri-miR-31 were placed under control of the liver-specific modified murine transthyretin promoter...
June 16, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28622647/endocrine-disrupting-activity-of-per-and-polyfluoroalkyl-substances-exploring-combined-approaches-of-ligand-and-structure-based-modeling
#12
Supratik Kar, Maria S Sepúlveda, Kunal Roy, Jerzy Leszczynski
Exposure to perfluorinated and polyfluoroalkyl substances (PFCs/PFASs), endocrine disrupting halogenated pollutants, has been linked to various diseases including thyroid toxicity in human populations across the globe. PFASs can compete with thyroxine (T4) for binding to the human thyroid hormone transport protein transthyretin (TTR) which may lead to reduce thyroid hormone levels leading to endocrine disrupting adverse effects. Environmental fate and endocrine-disrupting activity of PFASs has initiated several research projects, but the amount of experimental data available for these pollutants is limited...
June 9, 2017: Chemosphere
https://www.readbyqxmd.com/read/28615921/data-independent-acquisition-and-parallel-reaction-monitoring-mass-spectrometry-identification-of-serum-biomarkers-for-ovarian-cancer
#13
Navin Rauniyar, Gang Peng, TuKiet T Lam, Hongyu Zhao, Gil Mor, Kenneth R Williams
A data-independent acquisition (DIA)/parallel reaction monitoring (PRM) workflow was implemented to identify improved ovarian cancer biomarkers. Data-independent acquisition on ovarian cancer versus control sera and literature searches identified 50 biomarkers and indicated that apolipoprotein A-IV (ApoA-IV) is the most significantly differentially regulated protein. Parallel reaction monitoring with Targeted Ovarian Cancer Proteome Assay validated differential ApoA-IV expression and quantified 9 other biomarkers...
2017: Biomarker Insights
https://www.readbyqxmd.com/read/28611868/gastrointestinal-tract-amyloidosis-presenting-with-pneumatosis-intestinalis
#14
Vikram Raghunathan, David Louis, Baldeep Wirk
Pneumatosis intestinalis is a radiographic finding of gas pockets within the bowel wall. It can be associated with a range of diagnoses, but the most life-threatening causes are mesenteric ischemia, bowel necrosis, and bowel obstruction. Here we present the case of a patient with multiple myeloma who had pneumatosis intestinalis due to gastrointestinal amyloidosis, which is a rare manifestation of systemic amyloid disease. The patient had both transthyretin (ATTR) amyloidosis and acquired apolipoprotein serum amyloid A (AA) amyloidosis that are not usually seen in conjunction with multiple myeloma, which is most commonly associated with light-chain (AL) amyloidosis...
July 2017: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/28611125/design-and-rationale-of-the-phase-3-attr-act-clinical-trial-tafamidis-in-transthyretin-cardiomyopathy-clinical-trial
#15
Mathew S Maurer, Perry Elliott, Giampaolo Merlini, Sanjiv J Shah, Márcia Waddington Cruz, Alison Flynn, Balarama Gundapaneni, Carolyn Hahn, Steven Riley, Jeffrey Schwartz, Marla B Sultan, Claudio Rapezzi
Transthyretin amyloidosis is a rare, life-threatening disease resulting from aggregation and deposition of transthyretin amyloid fibrils in various tissues. There are 2 predominate phenotypic presentations of the disease: transthyretin familial amyloid polyneuropathy, which primarily affects the peripheral nerves, and transthyretin cardiomyopathy (TTR-CM), which primarily affects the heart. However, there is a wide overlap with symptoms at presentation and disease course being highly variable and influenced by the underlying transthyretin mutation, age of the affected individual, sex, and geographic location...
June 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/28609277/corino-de-andrade-disease-mechanisms-and-impact-on-reproduction
#16
REVIEW
Rita A Lopes, Teresa Coelho, Alberto Barros, Mário Sousa
Familial amyloid polyneuropathy was first described by Corino de Andrade in 1952 in Northern Portugal. It is a fatal autosomal dominant neurodegenerative disorder characterized by a progression of neurologic symptoms, beginning early in the reproductive life. The Transthyretin gene mutation originates a mutated protein that precipitates in the connective tissue as amyloid deposits. This disease is presently named Transthyretin-related hereditary amyloidosis. We performed an extensive review on this disease based on searches in Medical databases and in paper references...
June 1, 2017: JBRA Assisted Reproduction
https://www.readbyqxmd.com/read/28605421/diagnostic-sensitivity-of-abdominal-fat-aspiration-in-cardiac-amyloidosis
#17
Candida Cristina Quarta, Esther Gonzalez-Lopez, Janet A Gilbertson, Nichola Botcher, Dorota Rowczenio, Aviva Petrie, Tamer Rezk, Taryn Youngstein, Shameem Mahmood, Sajitha Sachchithanantham, Helen J Lachmann, Marianna Fontana, Carol J Whelan, Ashutosh D Wechalekar, Philip N Hawkins, Julian D Gillmore
Aims: Congo red staining of an endomyocardial biopsy is the diagnostic gold-standard in suspected cardiac amyloidosis (CA), but the procedure is associated with the risk, albeit small, of serious complications, and delay in diagnosis due to the requirement for technical expertise. In contrast, abdominal fat pad fine needle aspiration (FPFNA) is a simple, safe and well-established procedure in systemic amyloidosis, but its diagnostic sensitivity in patients with suspected CA remains unclear...
March 7, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28598686/how-your-ears-can-tell-what-is-hidden-in-your-heart-wild-type-transthyretin-amyloidosis-as-potential-cause-of-sensorineural-hearing-loss-inelderly-amylodeafness-pilot-study
#18
Emilie Béquignon, Aziz Guellich, Sophie Barthier, Marc Raynal, Virginie Prulière-Escabasse, Florence Canouï-Poitrine, André Coste, Thibaud Damy
BACKGROUND: Wild-type transthyretin amyloidosis (ATTRwt) is an age-related life-threatening condition. Prognosis is mainly dependent on cardiac involvement. Other organs and tissues may be affected. Their early recognition may increase awareness of physicians and positively affects the prognosis. Presbycusis is another age-related disorder. Whether this disease is associated to ATTRwt amyloidosis is unknown. METHODS: Sixteen consecutive patients with confirmed diagnosis of ATTRwt amyloidosis at the Mondor Amyloidosis Network, France, underwent otoscopy and audiological tests including pure tone audiometry, speech reception threshold and speech discrimination score...
June 9, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28598015/cutaneous-nerve-biomarkers-in-transthyretin-familial-amyloid-polyneuropathy
#19
Gigi J Ebenezer, Ying Liu, Daniel P Judge, Kelly Cunningham, Shaun Truelove, Noel D Carter, Blessan Sebastian, Kelly Byrnes, Michael Polydefkis
OBJECTIVE: To determine the utility of skin biopsies as a biomarker of disease severity in subjects with amyloid neuropathy. METHODS: Five groups of patients were studied: (1) transthyretin (TTR) familial amyloidotic polyneuropathy (FAP; n = 20), (2) TTR mutation carriers without peripheral neuropathy (TTR-noPN; n = 10), (3) healthy controls (n = 20), (4) diabetic neuropathy disease controls (n = 20), and (5) patients with light-chain (AL) amyloid (n = 2)...
June 9, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28590781/cognitive-impairment-in-liver-transplanted-patients-with-transthyretin-related-hereditary-amyloid-polyneuropathy
#20
Vanda Freitas Castro, Pedro Nascimento Alves, Ana Catarina Franco, Isabel Pavão Martins, Isabel Conceição
INTRODUCTION AND AIM: Hereditary transthyretin-related amyloidosis (ATTR-FAP) is characterized by a progressive neuropathy, cardiomyopathy, nephropathy and ocular disease. More than 90% of amyloidogenic transthyretin is produced by the liver; however, this protein is also synthesized in the choroid plexus. Although some patients have transitory neurologic events, the impact on cognition is still unknown. The aim was to study the cognitive performance of ATTR-FAP V30M patients with long disease course...
June 7, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
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