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NMR and encephalopathy

Emanuela Locci, Antonio Noto, Melania Puddu, Giulia Pomero, Roberto Demontis, Cristina Dalmazzo, Antonio Delogu, Vassilios Fanos, Ernesto d'Aloja, Paolo Gancia
Perinatal asphyxia is an event affecting around four million newborns worldwide. The 0.5 to 2 per 1000 of full term asphyxiated newborns suffer from hypoxic-ischemic encephalopathy (HIE), which is a frequent cause of death or severe disability and, as consequence, the most common birth injury claim for obstetrics, gynaecologists, and paediatricians. Perinatal asphyxia results from a compromised gas exchange that leads to hypoxemia, hypercapnia, and metabolic acidosis. In this work, we applied a metabolomics approach to investigate the metabolic profiles of urine samples collected from full term asphyxiated newborns with HIE undergoing therapeutic hypothermia (TH), with the aim of identifying a pattern of metabolites associated with HIE and to follow their modifications over time...
2018: PloS One
Vito Trinchieri, Luca Laghi, Beatrice Vitali, Carola Parolin, Ilaria Giusti, Daniela Capobianco, Paola Mastromarino, Claudio De Simone
Background: Variability in probiotics manufacturing may affect their properties, with potential implications for their efficacy and safety. This is of particular concern with probiotic products destined for use in patients with serious medical conditions, including human immunodeficiency virus (HIV) infection. The purpose of the study was to carry out a series of experiments comparing the properties of the US-made probiotic formulation originally commercialized under the brand name VSL#3® , with those of the Italian-made formulation now commercialized under the same name...
2017: Frontiers in Immunology
Yoshiki Shigemitsu, Hidekazu Hiroaki
Intrinsically disordered proteins (IDPs) are either completely unstructured or contain large disordered regions in their native state; they have drawn much attention in the field of molecular pathology. Some of them substantially tend to form protein self-assemblies, such as toxic or non-toxic aggregates and fibrils, and have been postulated to relate to diseases. These disease-related IDPs include Aβ(1-42) [Alzheimer's disease (AD)], Tau (AD and tauopathy), α-synuclein (Parkinson's disease) and p53 (cancer)...
January 1, 2018: Journal of Biochemistry
Ivana Biljan, Gregor Ilc, Janez Plavec
Prion diseases or transmissible spongiform encephalopathies constitute a group of fatal neurodegenerative diseases that can be of sporadic, genetic, or acquired origin. The central molecular event of prion diseases is the conformational conversion of the physiological cellular prion protein, PrP(C), into a disease-associated form known as prion or PrP(Sc). Spontaneous generation of prions in genetic prion diseases is caused by mutations in the human prion protein gene (PRNP). Understanding of the earliest conformational changes during misfolding of PrP(C) in genetic forms of prion diseases may benefit from detailed structural characterization of various human (Hu) PrP variants...
2017: Progress in Molecular Biology and Translational Science
Valerija Kovač, Blaž Zupančič, Gregor Ilc, Janez Plavec, Vladka Čurin Šerbec
In the brain of patients with transmissible spongiform encephalopathies, besides PrPSc aggregates, deposition of truncated PrP molecules was described. Jansen et al. reported two clinical cases with deposition of C-terminally truncated PrP, one of them ending with Tyr226. We have previously described the discovery of monoclonal antibody V5B2 that selectively recognizes this version of the prion protein, which we called PrP226*. Using monoclonal antibody V5B2 we showed that accumulation of PrP226* is characteristic for most types of human and animal TSEs...
February 26, 2017: Biochemical and Biophysical Research Communications
Mark J W McPhail, Sara Montagnese, Manuela Villanova, Hamza El Hadi, Piero Amodio, Mary M E Crossey, Roger Williams, I Jane Cox, Simon D Taylor-Robinson
To date urinary metabolic profiling has been applied to define a specific metabolic fingerprint of hepatocellular carcinoma on a background of cirrhosis. Its utility for the stratification of other complications of cirrhosis, such as hepatic encephalopathy (HE), remains to be established. Urinary proton nuclear magnetic resonance (1 H-NMR) spectra were acquired and NMR data from 52 patients with cirrhosis (35 male; 17 female, median (range) age [60 (18-81) years]) and 17 controls were compared. A sub-set of 45 patients (33 male; 12 female, [60 (18-90) years, median model for end stage liver disease (MELD) score 11 (7-27)]) were fully characterised by West-Haven criteria, Psychometric Hepatic Encephalopathy Score (PHES) and electroencephalogram (EEG), and defined as overt HE (OHE, n = 21), covert HE (cHE, n = 7) or no HE (n = 17)...
April 2017: Metabolic Brain Disease
Urška Slapšak, Giulia Salzano, Ladan Amin, Romany N N Abskharon, Gregor Ilc, Blaž Zupančič, Ivana Biljan, Janez Plavec, Gabriele Giachin, Giuseppe Legname
The cellular form of the prion protein (PrP(C)) is a highly conserved glycoprotein mostly expressed in the central and peripheral nervous systems by different cell types in mammals. A misfolded, pathogenic isoform, denoted as prion, is related to a class of neurodegenerative diseases known as transmissible spongiform encephalopathy. PrP(C) function has not been unequivocally clarified, and it is rather defined as a pleiotropic protein likely acting as a dynamic cell surface scaffolding protein for the assembly of different signaling modules...
October 14, 2016: Journal of Biological Chemistry
C E Ahearne, N M Denihan, B H Walsh, S N Reinke, L C Kenny, G B Boylan, D I Broadhurst, D M Murray
BACKGROUND: A 1H-NMR-derived metabolomic index based on early umbilical cord blood alterations of succinate, glycerol, 3-hydroxybutyrate and O-phosphocholine has shown potential for the prediction of hypoxic-ischaemic encephalopathy (HIE) severity. OBJECTIVE: To evaluate whether this metabolite score can predict 3-year neurodevelopmental outcome in infants with perinatal asphyxia and HIE, compared with current standard biochemical and clinical markers. METHODS: From September 2009 to June 2011, infants at risk of perinatal asphyxia were recruited from a single maternity hospital...
2016: Neonatology
Sunilkumar Puthenpurackal Narayanan, Divya Gopalakrishnan Nair, Daniel Schaal, Marisa Barbosa de Aguiar, Sabine Wenzel, Werner Kremer, Stephan Schwarzinger, Hans Robert Kalbitzer
Fatal neurodegenerative disorders termed transmissible spongiform encephalopathies (TSEs) are associated with the accumulation of fibrils of misfolded prion protein PrP. The noble gas xenon accommodates into four transiently enlarged hydrophobic cavities located in the well-folded core of human PrP(23-230) as detected by [(1)H, (15)N]-HSQC spectroscopy. In thermal equilibrium a fifth xenon binding site is formed transiently by amino acids A120 to L125 of the presumably disordered N-terminal domain and by amino acids K185 to T193 of the well-folded domain...
June 24, 2016: Scientific Reports
Luis Fonseca-Ornelas, Markus Zweckstetter
In a group of neurodegenerative diseases, collectively termed transmissible spongiform encephalopathies, the prion protein aggregates into β-sheet rich amyloid-like deposits. Because amyloid structure has been connected to different prion strains and cellular toxicity, it is important to obtain insight into the structural properties of prion fibrils. Using a combination of solution NMR spectroscopy, thioflavin-T fluorescence and electron microscopy we here show that within amyloid fibrils of a peptide containing residues 108-143 of the human prion protein [humPrP (108-143)]-the evolutionary most conserved part of the prion protein - residue H111 and S135 are in close spatial proximity and their interaction is critical for fibrillization...
August 2016: Protein Science: a Publication of the Protein Society
S Ottanelli, S Simeone, C Serena, M P Rambaldi, A Villanucci, K Tavella, G Amunni, F Mecacci, G Mello
INTRODUCTION: The occurrence of preeclampsia before the 20th week of gestation is rare and it has been associated with hydatidiform molar pregnancy. OBJECTIVES: We describe a case of first trimester eclampsia which occurred in a patient with hydatidiform mole. METHODS: Case report. RESULTS: A 16-year-old woman came to emergency service for abdominal pain and vaginal bleeding. She had been suffering of vomiting after meals and complaining for abdominal mass for 2months, without consulting her physician...
July 2012: Pregnancy Hypertension
Jiapu Zhang, Feng Wang, Subhojyoti Chatterjee
It was reported that buffalo is a low susceptibility species resisting to transmissible spongiform encephalopathies (TSEs) (same as rabbits, horses, and dogs). TSEs, also called prion diseases, are invariably fatal and highly infectious neurodegenerative diseases that affect a wide variety of species (except for rabbits, dogs, horses, and buffalo), manifesting as scrapie in sheep and goats; bovine spongiform encephalopathy (BSE or "mad-cow" disease) in cattle; chronic wasting disease in deer and elk; and Creutzfeldt-Jakob diseases, Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia, and Kulu in humans etc...
2016: Journal of Biomolecular Structure & Dynamics
Paola Scano, Antonella Rosa, Alessandra Incani, Caterina Maestrale, Cinzia Santucciu, Daniela Perra, Sarah Vascellari, Alessandra Pani, Ciriaco Ligios
While neurochemical metabolite modifications, determined by different techniques, have been diffusely reported in human and mice brains affected by transmissible spongiform encephalopathies (TSEs), this aspect has been little studied in the natural animal hosts with the same pathological conditions so far. Herein, we investigated, by high resolution (1)H NMR spectroscopy and multivariate statistical data analysis, the brain metabolite profile of sheep exposed to a scrapie agent in a naturally affected flock...
July 2015: Molecular BioSystems
Valerie Walker
Human adults produce around 1000 mmol of ammonia daily. Some is reutilized in biosynthesis. The remainder is waste and neurotoxic. Eventually most is excreted in urine as urea, together with ammonia used as a buffer. In extrahepatic tissues, ammonia is incorporated into nontoxic glutamine and released into blood. Large amounts are metabolized by the kidneys and small intestine. In the intestine, this yields ammonia, which is sequestered in portal blood and transported to the liver for ureagenesis, and citrulline, which is converted to arginine by the kidneys...
2014: Advances in Clinical Chemistry
Wenyi Hu, Xiaojie Cheng, Xinjian Ye, Liangcai Zhao, Yanan Huang, Huanle Zhu, Zhihan Yan, Xuebao Wang, Xiaojie Wang, Guanghui Bai, Hongchang Gao
BACKGROUND: Bilirubin encephalopathy (BE) is a severe neurologic sequelae induced by hyperbilirubinemia in newborns. However, the pathogenetic mechanisms underlying the clinical syndromes of BE remain ambiguous. Ex vivo (1)H nuclear magnetic resonance (NMR) spectroscopy was used to measure changes in the concentrations of cerebral metabolites in various brain areas of newborn 9-day-old rats subjected to bilirubin to explore the related mechanisms of BE. RESULTS: When measured 0...
2014: Molecular Brain
Takeshi Asano, Keiko Hirakawa, Kaoru Koike, Youkichi Ohno, Osamu Fujino
BACKGROUND: In acute encephalopathy, deterioration of the condition can be rapid, and early intervention is essential to prevent progression of the disease. However, in the acute period, differentiating acute encephalopathy from febrile seizures is difficult. Thus, an early diagnostic marker has been sought to enable early intervention. Proton nuclear magnetic resonance ((1)H NMR) spectroscopy is used to study the chemical characteristics of biological fluids such as cerebrospinal fluid (CSF)...
January 2015: Pediatric Research
Roger F Butterworth
Ineffective hepatic clearance of excess ammonia in the form of urea, as occurs in urea cycle enzymopathies (UCDs) and in liver failure, leads to increases in circulating and tissue concentrations of glutamine and a positive correlation between brain glutamine and the severity of neurological symptoms. Studies using 1H/13C Nuclear Magnetic Resonance (NMR) spectroscopy reveal increased de novo synthesis of glutamine in the brain in acute liver failure (ALF) but increases of synthesis rates per se do not correlate with either the severity of encephalopathy or brain edema...
September 2014: Molecular Genetics and Metabolism
Cristina R Bosoi, Claudia Zwingmann, Helen Marin, Christian Parent-Robitaille, Jimmy Huynh, Mélanie Tremblay, Christopher F Rose
BACKGROUND & AIMS: The pathogenesis of brain edema in patients with chronic liver disease (CLD) and minimal hepatic encephalopathy (HE) remains undefined. This study evaluated the role of brain lactate, glutamine and organic osmolytes, including myo-inositol and taurine, in the development of brain edema in a rat model of cirrhosis. METHODS: Six-week bile-duct ligated (BDL) rats were injected with (13)C-glucose and de novo synthesis of lactate, and glutamine in the brain was quantified using (13)C nuclear magnetic resonance spectroscopy (NMR)...
March 2014: Journal of Hepatology
Bogusław Habrat, Helena Baran-Furga, Halina Sienkiewicz-Jarosz, Jacek Sein Anand, Renata Poniatowska
Encephalopathy caused by manganese compounds used for illicit production of ephedrone (methcathinone) is described. The onset of disease could be observed after some months of regular intravenous use of ephedrone contaminated with manganese. In clinical picture dominate neurological signs and symptoms, mainly extrapyramidal syndromes: parkinsonism, tremor, muscle distonia, pro- and retropulsion. Some other symptoms may be observed: hypophonia or dysarthria, gain disturbances, impairment of precise movement, and micrographia...
2013: Przegla̧d Lekarski
Jiapu Zhang, Yuanli Zhang
Prion diseases, traditionally referred to as transmissible spongiform encephalopathies (TSEs), are invariably fatal and highly infectious neurodegenerative diseases that affect a wide variety of mammalian species, manifesting as scrapie in sheep and goats, bovine spongiform encephalopathy (BSE or mad-cow disease) in cattle, chronic wasting disease in deer and elk, and Creutzfeldt-Jakob diseases, Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia, and kulu in humans, etc. These neurodegenerative diseases are caused by the conversion from a soluble normal cellular prion protein (PrP(C)) into insoluble abnormally folded infectious prions (PrP(Sc)), and the conversion of PrP(C) to PrP(Sc) is believed to involve conformational change from a predominantly α-helical protein to one rich in β-sheet structure...
February 7, 2014: Journal of Theoretical Biology
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