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https://www.readbyqxmd.com/read/28719236/applying-an-artificial-neural-network-model-for-developing-a-severity-score-for-patients-with-hereditary-amyloid-polyneuropathy
#1
Shenia Novis, Felipe Machado, Victor B Costa, Debora Foguel, Marcia W Cruz, José Manoel de Seixas
Hereditary (familial) amyloid polyneuropathy (FAP) is a systemic disease that includes a sensorimotor polyneuropathy related to transthyretin (TTR) mutations. So far, a scale designed to classify the severity of this disease has not yet been validated. This work proposes the implementation of an artificial neural network (ANN) in order to develop a severity scale for monitoring the disease progression in FAP patients. In order to achieve this goal, relevant symptoms and laboratory findings were collected from 98 Brazilian patients included in THAOS - the Transthyretin Amyloidosis Outcomes Survey...
July 18, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28716239/acquired-transthyretin-amyloidosis-after-domino-liver-transplant-phenotypic-correlation-implication-of-liver-retransplantation
#2
Eli Muchtar, Martha Grogan, Surendra Dasari, Paul J Kurtin, Morie A Gertz
Reports of acquired ATTRm in domino liver transplant (DLT) recipients are infrequent and the approach to management is unknown. We describe two patients, an inherited ATTRm patient who underwent liver transplant and his liver recipient who developed acquired ATTRm 8years following transplantation. The clinical manifestations of amyloidosis were similar between patients and consisted of peripheral neuropathy, diarrhea and weight loss. Following liver transplantation, the DLT donor went into remission for 4years, but eventually progressed and died...
August 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28711731/insulin-chains-as-efficient-fusion-tags-for-prokaryotic-expression-of-short-peptides
#3
Ligang Deng, Xiaoying Xue, Cangjie Shen, Xiaohan Song, Chunyang Wang, Nan Wang
Insulin chains are usually expressed in Escherichia coli as fusion proteins with different tags, including various low molecular weight peptide tags. The objective of this study was to determine if insulin chains could facilitate the recombinant expression of other target proteins, with an emphasis on low molecular weight peptides. A series of short peptides were fused to mini-proinsulin, chain B or chain A, and induced for expression in Escherichia coli. All the tested peptides including glucagon-like peptide 1 (GLP-1), a C-terminal extended GLP-1, oxyntomodulin, enfuvirtide, linaclotide, and an unstructured artificial peptide were expressed with reasonable yields, identified by Tricine-SDS-PAGE and immunoblotting...
July 12, 2017: Protein Expression and Purification
https://www.readbyqxmd.com/read/28704493/evaluating-the-effect-of-mutations-and-ligand-binding-on-transthyretin-homotetramer-dynamics
#4
Tadeo E Saldaño, Giuseppe Zanotti, Gustavo Parisi, Sebastian Fernandez-Alberti
Native transthyretin (TTR) homotetramer dissociation is the first step of the fibrils formation process in amyloid disease. A large number of specific point mutations that destabilize TTR quaternary structure have shown pro-amyloidogenic effects. Besides, several compounds have been proposed as drugs in the therapy of TTR amyloidosis due to their TTR tetramer binding affinities, and therefore, contribution to its integrity. In the present paper we have explored key positions sustaining TTR tetramer dynamical stability...
2017: PloS One
https://www.readbyqxmd.com/read/28694632/late-onset-familial-amyloidotic-polyneuropathy-with-bence-jones-proteinuria-and-cardiomyopathy
#5
Sira Carrasco García de León, Amalia Hernández González, Carmen Orellana Alonso, Laura Burriel Lobo
Familial amyloidotic polyneuropathy is a genetically determined disease characterized by deposition of an anomalous transthyretin. A high index of suspicion is needed for this multisymptomatic and lethal disease to be diagnosed. The patient was a 70-year-old male examined due to hypesthesia in the hands and feet, plus difficulty walking. A neurophysiological study delivered the diagnosis of axonal sensorimotor polyneuropathy. He later developed cardiac symptoms and diarrhea. Urine laboratory analyses revealed a monoclonal spike of light chains (kappa)...
July 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28688921/plasma-retinol-concentration-is-mainly-driven-by-transthyretin-in-hemodialysis-patients
#6
Stanislas Bataille, Jean-François Landrier, Julien Astier, Sylvie Cado, Jérôme Sallette, Marianne Serveaux, Stéphane Burtey, Julien Cohen, Charlène Tournier, Franck Tourniaire, Patrice Darmon
OBJECTIVE: Micronutrients deficiencies in hemodialysis patients are due to low dietary intakes and intradialytic losses for hydrophilic micronutrients. Conversely, lipophilic nondialyzable compounds might accumulate because of a lack of elimination through renal metabolism or dialysis. Other compounds have complex metabolism: their concentration is not explained by these phenomenons. The aim of this study was to report plasma concentrations of lipophilic micronutrients in hemodialysis patients and to analyze if these concentrations were predictive of mortality...
July 6, 2017: Journal of Renal Nutrition
https://www.readbyqxmd.com/read/28686898/prenatal-transfer-of-decabromodiphenyl-ether-bde-209-results-in-disruption-of-the-thyroid-system-and-developmental-toxicity-in-zebrafish-offspring
#7
Zhihua Han, Yufei Li, Shenghu Zhang, Ninghui Song, Huaizhou Xu, Yao Dang, Chunsheng Liu, John P Giesy, Hongxia Yu
Decabromodiphenyl ether (BDE-209) was one of most widely-used polybrominated diphenyl ether (PBDE) flame retardants and is frequently detected in both abiotic and biotic samples from environment. However, knowledge of its transgenerational risks is limited. Here, 4-month-old zebrafish were exposed to various concentrations of BDE-209 (0, 3, 30 or 300μg/L) for 28days and spawned in clean water without BDE-209. Concentrations of triiodothyronine (T3) and thyroxine (T4) as well as expressions of genes involved in the hypothalamic-pituitary-thyroid (HPT) axis were measured in offspring after exposure of adult zebrafish to BDE-209...
June 23, 2017: Aquatic Toxicology
https://www.readbyqxmd.com/read/28683725/maldi-tof-ms-analysis-in-discovery-and-identification-of-serum-proteomic-patterns-of-ovarian-cancer
#8
Agata Swiatly, Agnieszka Horala, Joanna Hajduk, Jan Matysiak, Ewa Nowak-Markwitz, Zenon J Kokot
BACKGROUND: Due to high mortality and lack of efficient screening, new tools for ovarian cancer (OC) diagnosis are urgently needed. To broaden the knowledge on the pathological processes that occur during ovarian cancer tumorigenesis, protein-peptide profiling was proposed. METHODS: Serum proteomic patterns in samples from OC patients were obtained using matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF). Eighty nine serum samples (44 ovarian cancer and 45 healthy controls) were pretreated using solid-phase extraction method...
July 6, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28679600/sural-nerve-injury-in-familial-amyloid-polyneuropathy-mr-neurography-vs-clinicopathologic-tools
#9
Jennifer Kollmer, Felix Sahm, Ute Hegenbart, Jan C Purrucker, Christoph Kimmich, Stefan O Schönland, Ernst Hund, Sabine Heiland, John M Hayes, Arnt V Kristen, Christoph Röcken, Mirko Pham, Martin Bendszus, Markus Weiler
OBJECTIVE: To detect and quantify lesions of the small-caliber sural nerve (SN) in symptomatic and asymptomatic transthyretin familial amyloid polyneuropathy (TTR-FAP) by high-resolution magnetic resonance neurography (MRN) in correlation with electrophysiologic and histopathologic findings. METHODS: Twenty-five patients with TTR-FAP, 10 asymptomatic carriers of the mutated transthyretin gene (mutTTR), and 35 age- and sex-matched healthy controls were prospectively included in this cross-sectional case-control study...
July 5, 2017: Neurology
https://www.readbyqxmd.com/read/28679024/multi-pronged-proteomic-analysis-to-study-the-glioma-pathobiology-using-cerebrospinal-fluid-samples
#10
Nikita Gahoi, Darpan Malhotra, Aliasgar Moiyadi, Santosh G Varma, Mayuri N Gandhi, Sanjeeva Srivastava
PURPOSE: Gliomas are one of the most aggressive and lethal brain tumors arising from neoplastic transformation of astrocytes and oligodendrocytes. In this study, we have carried out a comprehensive quantitative analysis of proteome level differences in cerebrospinal fluid (CSF) across different grades of gliomas for a better understanding of glioma pathobiology. EXPERIMENTAL DESIGN: Glioma patients were diagnosed by radiology and histochemistry- based analyses. Differential proteomic analysis of high (n = 12) and low (n = 8) grade gliomas, and control (n = 3) samples was performed by using two complementary quantitative proteomic approaches; 2D-DIGE and iTRAQ...
July 5, 2017: Proteomics. Clinical Applications
https://www.readbyqxmd.com/read/28678039/familial-amyloid-polyneuropathy
#11
David Adams, Cécile Cauquil, Céline Labeyrie
PURPOSE OF REVIEW: Transthyretin familial amyloid polyneuropathy is the most disabling hereditary polyneuropathy of adult onset because of a point mutation of transthyretin gene. This review updates our knowledge about natural history of the disease, phenotypes, diagnosis tools for small and large fibers involvement, expert's consensus for both symptomatic and asymptomatic follow-up, and treatment's research. RECENT FINDINGS: Access to TTR gene sequencing permit diagnosis and first reports of the disease in nonendemic countries (EU countries, United States, China, India)...
July 3, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28675715/identification-of-potential-biomarkers-for-systemic-lupus-erythematosus-diagnosis-using-two-dimensional-differential-gel-electrophoresis-2d-dige-and-mass-spectrometry
#12
Tamara Aparecida Reis Ferreira, Hélida Monteiro de Andrade, Paulo Madureira de Pádua, Maria das Graças Carvalho, Simone da Fonseca Pires, Ivana Helena Rocha Oliveira, Bruna Soares Souza Lima, Luis Carlos Fialho Júnior, Walter Batista Cicarini, Donat Alexander Chapeourouge, Jonas Henrique Perales, Tânia Mara Pinto Dabés Guimarães, Vicente de Paulo Coelho Peixoto de Toledo
Systemic lupus erythematosus (SLE) is an autoimmune disease of the connective tissue with a large spectrum of clinical manifestations. Immune deregulation leads to autoantibody and immune complexes overproduction, complement activation, and persistent tissue inflammation. Considering that the current diagnosis depends on the interpretation of the complex criteria established by the American College of Rheumatology and that the disease course is characterized by unpredictable activations and remissions, each patient develops different manifestations, and therefore, the discovery of specific biomarkers is urgently required...
July 4, 2017: Autoimmunity
https://www.readbyqxmd.com/read/28675284/-transthyretin-related-hereditary-amyloidosis
#13
Julien Vionnet, Manuel Pascual, Thierry Kuntzer, Patrick Yerly, Darius Moradpour
Transthyretin-related hereditary amyloidosis (ATTR) is a progressive and potentially lethal genetic disorder, transmitted as an autosomal dominant trait. Tissue injury is induced by amyloid fibrils consisting of mutated transthyretin. The symptomatology and clinical course of ATTR is highly variable but typically causes peripheral polyneuropathy and autonomic dysfunction, leading to death within 10 years. As transthyretin is produced mainly in the liver, liver transplantation was the first successful therapeutic approach...
August 31, 2016: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28667533/hydrolysis-and-dissolution-of-amyloids-by-catabodies
#14
Ville V Meretoja, Sudhir Paul, Stephanie A Planque
Catalytic antibodies (catabodies) hold potential for superior immunotherapy because of their turnover capability and no or minimal induction of inflammatory responses. Catabodies neutralize and remove target antigens more potently than conventional antibodies. Depending on the catalytic rate constant, a single catabody molecule degrades thousands to millions of target molecules over its useful lifespan, whereas conventional antibodies only form reversibly associated, stoichiometric complexes with the target...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28660543/therapeutic-strategies-targeting-inherited-cardiomyopathies
#15
REVIEW
Kenneth Varian, W H Wilson Tang
PURPOSE OF REVIEW: Cardiomyopathies due to genetic mutations are a heterogeneous group of disorders that comprise diseases of contractility, myocardial relaxation, and arrhythmias. Our goal here is to discuss a limited list of genetically inherited cardiomyopathies and the specific therapeutic strategies used to treat them. RECENT FINDINGS: Research into the molecular pathophysiology of the development of these cardiomyopathies is leading to the development of novel treatment approaches...
June 28, 2017: Current Heart Failure Reports
https://www.readbyqxmd.com/read/28660252/abnormal-expression-of-homeobox-genes-and-transthyretin-in-c9orf72-expansion-carriers
#16
NiCole A Finch, Xue Wang, Matthew C Baker, Michael G Heckman, Tania F Gendron, Kevin F Bieniek, Joanne Wuu, Mariely DeJesus-Hernandez, Patricia H Brown, Jeannie Chew, Karen R Jansen-West, Lillian M Daughrity, Alexandra M Nicholson, Melissa E Murray, Keith A Josephs, Joseph E Parisi, David S Knopman, Ronald C Petersen, Leonard Petrucelli, Bradley F Boeve, Neill R Graff-Radford, Yan W Asmann, Dennis W Dickson, Michael Benatar, Robert Bowser, Kevin B Boylan, Rosa Rademakers, Marka van Blitterswijk
OBJECTIVE: We performed a genome-wide brain expression study to reveal the underpinnings of diseases linked to a repeat expansion in chromosome 9 open reading frame 72 (C9ORF72). METHODS: The genome-wide expression profile was investigated in brain tissue obtained from C9ORF72 expansion carriers (n = 32), patients without this expansion (n = 30), and controls (n = 20). Using quantitative real-time PCR, findings were confirmed in our entire pathologic cohort of expansion carriers (n = 56) as well as nonexpansion carriers (n = 31) and controls (n = 20)...
August 2017: Neurology. Genetics
https://www.readbyqxmd.com/read/28652559/expression-of-thyroid-hormone-regulator-genes-in-the-yolk-sac-membrane-of-the-developing-chicken-embryo
#17
Hanny Cho Too, Mitsuhiro Shibata, Masato Yayota, Veerle M Darras, Atsushi Iwasawa
Thyroid hormones (THs) are essential for the correct development of nearly every structure in the body from the very early stages of development, yet the embryonic thyroid gland is not functional at these stages. To clarify the roles of the egg yolk as a source of THs, the TH content in the yolk and the expression of TH regulator genes in the yolk sac membrane were evaluated throughout the 21-day incubation period of chicken embryos. The yolk TH content (22.3 ng triiodothyronine and 654.7 ng thyroxine per total yolk on day 4 of incubation) decreased almost linearly along with development...
June 25, 2017: Journal of Reproduction and Development
https://www.readbyqxmd.com/read/28648829/novel-pharmacotherapies-for-cardiac-amyloidosis
#18
REVIEW
Kevin M Alexander, Avinainder Singh, Rodney H Falk
Amyloidosis refers to a range of protein misfolding disorders that can cause organ dysfunction through progressive fibril deposition. Cardiac involvement often leads to significant morbidity and mortality and increasingly has been recognized as an important cause of heart failure. The two main forms of cardiac amyloidosis, light chain (AL) and transthyretin (ATTR) amyloidosis, have distinct mechanisms of pathogenesis. Recent insights have led to the development of novel pharmacotherapies with the potential to significantly impact each disease...
June 22, 2017: Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28647518/increasing-the-accuracy-of-proteomic-typing-by-decellularisation-of-amyloid-tissue-biopsies
#19
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
#20
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
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