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Ceruloplasmin

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https://www.readbyqxmd.com/read/29341979/wilson-s-disease-in-children-a-position-paper-by-the-european-society-for-paediatric-gastroenterology-hepatology-and-nutrition-committee
#1
Piotr Socha, Wojciech Janczyk, Anil Dhawan, Ulrich Baumann, Lorenzo D'Antiga, Stuart Tanner, Raffaele Iorio, Pietro Vajro, Roderick Houwen, Björn Fischler, Antal Dezsofi, Nedim Hadzic, Loreto Hierro, Jörg Jahnel, Valérie McLin, Valerio Nobili, Francoise Smets, Henkjan J Verkade, Dominique Debray
BACKGROUND: Clinical presentations of Wilson's disease (WD) in childhood ranges from asymptomatic liver disease to cirrhosis or acute liver failure, while neurological and psychiatric symptoms are rare. The basic diagnostic approach includes serum ceruloplasmin and 24h-urinary copper excretion. Final diagnosis of WD can be established using a diagnostic scoring system based on symptoms, biochemical tests assessing copper metabolism and molecular analysis of mutations in the ATP7B gene...
October 26, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29332043/serum-non-ceruloplasmin-non-albumin-copper-elevation-in-mild-cognitive-impairment-and-dementia-due-to-alzheimer-s-disease-a-case-control-study
#2
Luca Rozzini, Francesco Lanfranchi, Andrea Pilotto, Simona Catalani, Maria Enrica Gilberti, Matteo Paganelli, Pietro Apostoli, Alessandro Padovani
Several studies showed high serum copper levels in Alzheimer's disease (AD). The present study applied a newly developed method to detect serum copper free from proteins (free-Cu). Forty-four patients affected by dementia due to AD, thirty-six patients affected by mild cognitive impairment (MCI) due to AD, and twenty-eight healthy controls underwent clinical, cognitive, and MRI assessment. The new method showed higher free-Cu concentrations in MCI and dementia due to AD compared to controls (p < 0.0001)...
2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29331561/long-term-outcome-of-neurological-wilson-s-disease
#3
Harald Hefter, Osman Tezayak, Dietmar Rosenthal
INTRODUCTION: Aim of the study was to characterize the clinical spectrum of long-term treated patients with Wilson's disease (WD) and to identify risk factors influencing long-term outcome. METHODS: In a cross-sectional study 30 WD-patients being treated for at least 2.5 and up to 31 years underwent a detailed clinical investigation, scoring of clinical findings yielding 7 motor and 3 non-motor subscores as well as laboratory testing. A factor analysis of these subscores and laboratory parameters was performed to detect those items with the highest influence on outcome, an ANOVA and subgroup analysis tested the influence of age, age at onset of diagnosis and duration of treatment on outcome...
January 4, 2018: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29329239/fine-regulation-of-neutrophil-oxidative-status-and-apoptosis-by-ceruloplasmin-and-its-derivatives
#4
Ekaterina A Golenkina, Galina M Viryasova, Svetlana I Galkina, Tatjana V Gaponova, Galina F Sud'ina, Alexey V Sokolov
Timely neutrophil apoptosis is an essential part of the resolution phase of acute inflammation. Ceruloplasmin, an acute-phase protein, which is the predominant copper-carrying protein in the blood, has been suggested to have a marked effect on neutrophil life span. The present work is a comparative study on the effects of intact holo-ceruloplasmin, its copper-free (apo-) and partially proteolyzed forms, and synthetic free peptides RPYLKVFNPR (883-892) and RRPYLKVFNPRR (882-893) on polymorphonuclear leukocyte (PMNL, neutrophil) oxidant status and apoptosis...
January 12, 2018: Cells
https://www.readbyqxmd.com/read/29324775/the-optimal-threshold-of-serum-ceruloplasmin-in-the-diagnosis-of-wilson-s-disease-a-large-hospital-based-study
#5
Rong Xu, Yong-Fang Jiang, Yong-Hong Zhang, Xu Yang
BACKGROUND AND AIMS: A ceruloplasmin (CP) concentration <200 mg/L is conventionally considered as one of the major diagnostic criteria for Wilson's disease (WD). However, the diagnostic accuracy of this threshold has never been investigated in a sufficiently large group of patients. This study aims to present the results of serum CP measurements in various patients and to identify the optimal cutoff value of CP for the diagnosis of WD. MATERIALS AND METHODS: We identified patients whose CP levels were evaluated from January 1, 2016 to December 31, 2016 using a laboratory information database...
2018: PloS One
https://www.readbyqxmd.com/read/29322430/serum-copper-homeostasis-in-hypertensive-intracerebral-hemorrhage-and-its-clinical-significance
#6
Ming Han, Shan Ding, Yuan Zhang, Zhexuan Lin, Kangsheng Li
This study was to investigate the alterations of serum copper homeostasis after hypertensive intracerebral hemorrhage (ICH), which is not yet clear. We recruited 85 hypertensive ICH patients and determined their serum levels of total copper (TCu), small molecule copper (SMC), and ceruloplasmin (Cp). Sera from 32 healthy persons and 12 primary hypertension patients were collected and analyzed as well. Serum TCu levels in ICH patients were tested at three time points (on admission, day 3, and day 7) and found to be higher than that in hypertension patients (p < 0...
January 11, 2018: Biological Trace Element Research
https://www.readbyqxmd.com/read/29296776/intestinal-hephaestin-potentiates-iron-absorption-in-weanling-adult-and-pregnant-mice-under-physiological-conditions
#7
Caglar Doguer, Jung-Heun Ha, Sukru Gulec, Chris D Vulpe, Gregory J Anderson, James F Collins
Regulation of intestinal iron absorption is crucial to maintain body iron levels because humans have no regulated iron-excretory system. Elucidating molecular events that mediate intestinal iron transport is thus important for the development of therapeutic approaches to modify iron absorption in pathological states. The process of iron uptake into duodenal enterocytes is relatively well understood, but less is known about the functional coupling between the iron exporter ferroportin 1 and the basolateral membrane iron oxidase hephaestin (Heph)...
July 25, 2017: Blood Advances
https://www.readbyqxmd.com/read/29295524/dietary-copper-reduces-the-hepatotoxicity-of-epigallocatechin-3-gallate-in-mice
#8
Najeeb Ahmed Kaleri, Kang Sun, Le Wang, Jin Li, Wenzheng Zhang, Xuan Chen, Xinghui Li
We developed Cu-deficient, -sufficient and -super nutrition mice models by feeding them with diet containing 1.68, 11.72 or 51.69 mg of Cu/kg for 28 days, respectively. Then, the mice were treated to (-)-epigallocatechin-3-gallate (EGCG, 750 mg/kg BW) by oral in order to assess the acute toxicity of the drug. Following EGCG treatment, the survival rates were 12.5%, 50% and 100% in the Cu-deficient, -sufficient and Cu-super nutrition groups of mice, respectively. Cu level and ceruloplasmin activity in serum were significantly increased with the increase of dietary Cu...
December 23, 2017: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/29280967/biomarkers-for-pulmonary-inflammation-and-fibrosis-and-lung-ventilation-function-in-chinese-occupational-refractory-ceramic-fibers-exposed-workers
#9
Xiaojun Zhu, Yishuo Gu, Wenjun Ma, Panjun Gao, Mengxuan Liu, Pei Xiao, Hongfei Wang, Juan Chen, Tao Li
Refractory ceramic fibers (RCFs) can cause adverse health effects on workers' respiratory system, yet no proper biomarkers have been used to detect early pulmonary injury of RCFs-exposed workers. This study assessed the levels of two biomarkers that are related to respiratory injury in RCFs-exposed workers, and explored their relations with lung function. The exposure levels of total dust and respirable fibers were measured simultaneously in RCFs factories. The levels of TGF-β1 and ceruloplasmin (CP) increased with the RCFs exposure level (p < 0...
December 27, 2017: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/29235214/the-effect-of-administration-of-copper-nanoparticles-to-chickens-in-their-drinking-water-on-the-immune-and-antioxidant-status-of-the-blood
#10
Katarzyna Ognik, Iwona Sembratowicz, Ewelina Cholewińska, Jan Jankowski, Krzysztof Kozłowski, Jerzy Juśkiewicz, Zenon Zduńczyk
The aim of this experiment was to determine what dosage of copper (Cu) nanoparticles, added to a standard dietary supplementation with copper sulfate, would improve antioxidant and immune defense in chickens. The experiment was conducted with 126 broiler chickens assigned to seven treatments with three pens per treatment and six broiler chickens per pen. The basal-diet treatment did not receive Cu nanoparticles (nano-Cu) and as shown by analyses it was Cu-deficient (-29% vs. National Research Council (NRC) recommendations; 5...
December 12, 2017: Animal Science Journal, Nihon Chikusan Gakkaihō
https://www.readbyqxmd.com/read/29234088/tear-proteome-analysis-in-ocular-surface-diseases-using-label-free-lc-ms-ms-and-multiplexed-microarray-biomarker-validation
#11
Javier Soria, Arantxa Acera, Jesús Merayo-LLoves, Juan A Durán, Nerea González, Sandra Rodriguez, Nikitas Bistolas, Soeren Schumacher, Frank F Bier, Harald Peter, Walter Stöcklein, Tatiana Suárez
We analyzed the tear film proteome of patients with dry eye (DE), meibomian gland dysfunction (MGD), and normal volunteers (CT). Tear samples were collected from 70 individuals. Of these, 37 samples were analyzed using spectral-counting-based LC-MS/MS label-free quantitation, and 33 samples were evaluated in the validation of candidate biomarkers employing customized antibody microarray assays. Comparative analysis of tear protein profiles revealed differences in the expression levels of 26 proteins, including protein S100A6, annexin A1, cystatin-S, thioredoxin, phospholipase A2, antileukoproteinase, and lactoperoxidase...
December 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29229441/does-dietary-insect-meal-affect-the-fish-immune-system-the-case-of-mealworm-tenebrio-molitor-on-european-sea-bass-dicentrarchus-labrax
#12
M A Henry, L Gasco, S Chatzifotis, G Piccolo
Feeding small European sea bass, Dicentrarchus labrax, for 6 weeks with Tenebrio molitor larval meal showed significant anti-inflammatory responses (ceruloplasmin, myeloperoxidase and nitric oxide). Serum bacteriolytic activity against a Gram negative bacterium was not significantly affected by dietary Tenebrio, while both lysozyme antibacterial activity and serum trypsin inhibition usually linked to the anti-parasite activity of the fish, were significantly enhanced. The latter may be due to the similarities in the composition of the exoskeleton of parasites and insects that may therefore act as an immunostimulant potentially increasing the anti-parasitic activity...
December 8, 2017: Developmental and Comparative Immunology
https://www.readbyqxmd.com/read/29225221/a-new-kinetic-automated-assay-to-determine-the-ferroxidase-activity-of-ceruloplasmin
#13
Salim Neselioglu, Merve Ergin, Ozcan Erel
A new kinetic and automated assay was developed to determine ceruloplasmin ferroxidase activity. Ferrous ions are turned into ferric ions via catalytic activity of the ferroxidase enzyme. Acetohydroxamic acid, a chromogen, forms a colored complex with ferric ions. This reaction was measured kinetically. Significant and strong correlations were obtained between the new acetohydroxamic method and the p-phenylenediamine oxidase (r = 0.988, p <0.001), o-dianisidine oxidase (r = 0.981, p <0.001), norfloxacine oxidase (r = 0...
2017: Analytical Sciences: the International Journal of the Japan Society for Analytical Chemistry
https://www.readbyqxmd.com/read/29203265/a-low-blood-copper-concentration-is-a-co-morbidity-burden-factor-in-parkinson-s-disease-development
#14
Ekaterina Y Ilyechova, Iren V Miliukhina, Iurii A Orlov, Zamira M Muruzheva, Ludmila V Puchkova, Marina N Karpenko
Parkinson's disease (PD) patients are often characterized by copper dyshomeostasis, which is responsible for ROS formation and fibrillogenesis. However, the relationships between copper metabolism and PD development are unclear. In this study in 50 patients with PD (pPD) and 50 age-matched healthy individuals, the serum total copper concentration, oxidase activity, ceruloplasmin and SOD3 protein concentrations were measured; and amount of copper atoms per ceruloplasmin molecule was calculated. These parameters were lower in pPD relatively to healthy volunteers...
December 1, 2017: Neuroscience Research
https://www.readbyqxmd.com/read/29195219/nanoconjugates-of-ferrocene-and-carbon-encapsulated-iron-nanoparticles-as-sensing-platforms-for-voltammetric-determination-of-ceruloplasmin-in-blood
#15
Jakub P Sęk, Artur Kasprzak, Michał Bystrzejewski, Magdalena Poplawska, Waldemar Kaszuwara, Zbigniew Stojek, Anna M Nowicka
The nanoparticles comprising of iron core and carbon shell were decorated with ferrocene derivatives: ferrocenecarboxaldehyde (Fc-1) and ferrocenecarboxaldehyde oxime (Fc-2). A microdrop of suspension of the nanoconjugate was placed on a glassy-carbon electrode to prepare the recognition/sensing layer. Drying and purification of the sensing layer resulted in a well-defined and stable square-wave voltammogram of the ferrocene moiety. The height of the voltammetric peak increased in the presence of ceruloplasmin...
November 23, 2017: Biosensors & Bioelectronics
https://www.readbyqxmd.com/read/29183916/ceruloplasmin-replacement-therapy-ameliorates-neurological-symptoms-in-a-preclinical-model-of%C3%A2-aceruloplasminemia
#16
Alan Zanardi, Antonio Conti, Marco Cremonesi, Patrizia D'Adamo, Enrica Gilberti, Pietro Apostoli, Carlo Vittorio Cannistraci, Alberto Piperno, Samuel David, Massimo Alessio
Aceruloplasminemia is a monogenic disease caused by mutations in the ceruloplasmin gene that result in loss of protein ferroxidase activity. Ceruloplasmin plays a role in iron homeostasis, and its activity impairment leads to iron accumulation in liver, pancreas, and brain. Iron deposition promotes diabetes, retinal degeneration, and progressive neurodegeneration. Current therapies mainly based on iron chelation, partially control systemic iron deposition but are ineffective on neurodegeneration. We investigated the potential of ceruloplasmin replacement therapy in reducing the neurological pathology in the ceruloplasmin-knockout (CpKO) mouse model of aceruloplasminemia...
November 28, 2017: EMBO Molecular Medicine
https://www.readbyqxmd.com/read/29177316/rat-ceruloplasmin-a-new-labile-copper-binding-site-and-zinc-copper-mosaic
#17
V R Samygina, A V Sokolov, G Bourenkov, T R Schneider, V A Anashkin, S O Kozlov, N N Kolmakov, V B Vasilyev
Ceruloplasmin (Cp) is a copper-containing multifunctional oxidase of plasma, an antioxidant, an acute-phase protein and a free radical scavenger. The structural organization of Cp causes its sensitivity to proteolysis and ROS (reactive oxygen species), which can alter some of the important Cp functions. Elucidation of the orthorhombic crystal structure of rat Cp at 2.3 Å resolution revealed the basis for stronger resistance of rat Cp to proteolysis and a new labile copper binding site. The presence of this site appears as a very rare and distinctive feature of rat Cp as was shown by sequence alignment of ceruloplasmin, hephaestin and zyklopen in the Deuterostomia taxonomic group...
November 27, 2017: Metallomics: Integrated Biometal Science
https://www.readbyqxmd.com/read/29173477/copper-dyshomeostasis-in-wilson-disease-and-alzheimer-s-disease-as-shown-by-serum-and-urine-copper-indicators
#18
Rosanna Squitti, Roberta Ghidoni, Ilaria Simonelli, Irena D Ivanova, Nicola Antonio Colabufo, Massimo Zuin, Luisa Benussi, Giuliano Binetti, Emanuele Cassetta, Mauro Rongioletti, Mariacristina Siotto
Abnormal handling of copper is the cause of Wilson disease (WD), a rare disorder typified by increased levels in plasma copper not-bound to ceruloplasmin (nCp-Cu, also known as 'free' copper). In Alzheimer's disease (AD), meta-analyses show that copper decreases in brain but increases in serum, due to the nCp Cu component increase. Despite the similarities, a direct comparison of copper biological status in the two diseases has never been carried out. To fill this gap, we evaluated serum copper, ceruloplasmin, nCp-Cu and Cu:Cp in 385 CE and 336 healthy controls previously investigated that were compared with 9 newly diagnosed WD patients...
January 2018: Journal of Trace Elements in Medicine and Biology
https://www.readbyqxmd.com/read/29169133/alterations-of-mitochondrial-antioxidant-indexes-and-apoptosis-in-duck-livers-caused-by-molybdenum-or-and-cadmium
#19
Xueyan Dai, Chenghong Xing, Huabin Cao, Junrong Luo, Tiancheng Wang, Ping Liu, Xiaoquan Guo, Guoliang Hu, Caiying Zhang
Cadmium (Cd) and high Molybdenum (Mo) can lead to adverse reactions on animals, but the co-induced toxicity of Mo and Cd to liver in ducks was not well understood. To investigate the co-induced toxic effects of Mo combined with Cd on mitochondrial oxidative stress and apoptosis in duck livers. 240 healthy 11-day-old ducks were randomly divided into 6 groups (control, LMo group, HMo group, Cd group, LMoCd group and HMoCd group). After being treated for 30, 60, 90 and 120 days, liver mitochondrial antioxidant indexes, ceruloplasmin (CP), metallothionein (MT), Bak-1 and Caspase-3 genes mRNA expression levels, and ultrastructural changes were evaluated...
November 14, 2017: Chemosphere
https://www.readbyqxmd.com/read/29152905/the-gait-translational-control-system
#20
REVIEW
Abul Arif, Peng Yao, Fulvia Terenzi, Jie Jia, Partho Sarothi Ray, Paul L Fox
The interferon (IFN)-γ-activated inhibitor of translation (GAIT) system directs transcript-selective translational control of functionally related genes. In myeloid cells, IFN-γ induces formation of a multiprotein GAIT complex that binds structural GAIT elements in the 3'-untranslated regions (UTRs) of multiple inflammation-related mRNAs, including ceruloplasmin and VEGF-A, and represses their translation. The human GAIT complex is a heterotetramer containing glutamyl-prolyl tRNA synthetase (EPRS), NS1-associated protein 1 (NSAP1), ribosomal protein L13a (L13a), and glyceraldehyde-3-phosphate dehydrogenase (GAPDH)...
November 20, 2017: Wiley Interdisciplinary Reviews. RNA
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