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https://www.readbyqxmd.com/read/28435998/a-6-year-old-boy-with-wilson-disease-a-diagnostic-dilemma
#1
Ramaswamy Ganesh, N Suresh, T Vasanthi, Malathi Sathiyasekaran, R Thulasiraman
A 6-year-old boy presented with 2 months history of progressive abdominal distension and jaundice. He was deeply icteric with ascites, hepatosplenomegaly, hyperbilirubinemia, raised transaminases, and coagulopathy. Viral markers and slit lamp examination for Kayser-Fleischer ring were negative. Serum ceruloplasmin and 24-h urinary copper post-D-pencillamine challenge were normal. Anti-smooth muscle antibody was positive 1:20, and liver biopsy showed micronodular cirrhosis with abundant Mallory hyaline and stainable copper deposits...
April 24, 2017: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/28435317/systemic-alterations-in-plasma-proteins-from-women-with-chronic-widespread-pain-compared-to-healthy-controls-a-proteomic-study
#2
Karin Wåhlén, Patrik Olausson, Anders Carlsson, Nazdar Ghafouri, Björn Gerdle, Bijar Ghafouri
Chronic widespread pain (CWP) is a complex pain condition that is difficult to treat. The prevalence of CWP approximates ~10% of the general population, with higher prevalence in women. Lack of understanding of molecular mechanisms has been a challenge for diagnosis and treatment of chronic pain. The aim of this study was to explore the systemic protein changes in CWP compared to those in healthy controls (CON). By applying 2-dimensional gel electrophoresis, we analyzed the protein pattern of plasma samples from women with CWP (n=16) and healthy women (n=23)...
2017: Journal of Pain Research
https://www.readbyqxmd.com/read/28433714/effects-of-dietary-supplementation-with-fenugreek-seeds-alone-or-in-combination-with-probiotics-on-gilthead-seabream-sparus-aurata-l-skin-mucosal-immunity
#3
F A Guardiola, A Bahi, A Bakhrouf, M A Esteban
Despite increasing interest in modulating the immune response of fish, providing a combination of probiotics and herbal immunostimulants in aquafeed has rarely has been studied. The effects on gilthead seabream (Sparus aurata L.) of the dietary administration of fenugreek (Trigonella foenum graecum) seeds alone (FE), or combined with one of the following probiotic strains: Bacillus licheniformis (FEBL), Lactobacillus plantarum (FELP) or Bacillus subtilis (FEBS) were evaluated. Fish were fed a control or one of the supplemented diets for 3 weeks...
April 19, 2017: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/28433109/pathogenesis-of-wilson-disease
#4
Ivo Florin Scheiber, Radan Brůha, Petr Dušek
Wilson disease is an autosomal-recessive disorder originating from a genetic defect in the copper-transporting ATPase ATP7B that is required for biliary copper secretion and loading of ceruloplasmin with copper. Impaired ATP7B function in Wilson disease results in excessive accumulation of copper in liver, brain, and other tissues. Toxic copper deposits may induce oxidative stress, modify expression of genes, directly inhibit proteins, and impair mitochondrial function, leading to hepatic, neuropsychiatric, renal, musculoskeletal, and other symptoms...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28433100/diagnosis-of-wilson-disease
#5
Peter Ferenci
Clinical presentation of Wilson disease can vary widely; therefore diagnosis is not always straightforward. Wilson disease is not just a disease of children and young adults, but may present at any age. The key features of Wilson disease are liver disease and cirrhosis, neuropsychiatric disturbances, Kayser-Fleischer rings, and acute episodes of hemolysis, often in association with acute liver failure. Diagnosis is particularly difficult in children and in adults presenting with active liver disease. None of the available laboratory tests is perfect and may not be specific for Wilson disease...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28433098/wilson-disease-in-children
#6
Eve A Roberts, Piotr Socha
Wilson disease (WD) is an inherited disorder mainly of hepatocellular copper disposition, due to dysfunction of the Wilson ATPase, a P1B-ATPase encoded by the gene ATP7B. In children, as in older age brackets, clinical disease is highly diverse. Although hepatic disease is the common presentation in children/adolescents, neurologic, psychiatric, and hematologic clinical presentations do occur. Very young children may have clinically evident liver disease due to WD. Early diagnosis, preferably when the child/adolescent is asymptomatic, is most likely to result in near-normal longevity with generally good health so long as the patient tolerates effective medication, is adherent to the lifelong treatment regimen, and has consistent access to the medication...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28428350/targeted-inactivation-of-copper-transporter-atp7b-in-hepatocytes-causes-liver-steatosis-and-obesity-in-mice
#7
Abigael Muchenditsi, Haojun Yang, James P Hamilton, Lahari Koganti, Franck Housseau, Lisa Aronov, Hongni Fan, Hannah Pierson, Ashima Bhattacharjee, Robert C Murphy, Cynthia L Sears, James J Potter, Clavia Ruth Wooton-Kee, Svetlana Lutsenko
The copper transporter ATP7B is essential for mammalian copper homeostasis. Mutations in ATP7B result in copper accumulation, especially in the liver, and cause Wilson disease (WD). The major role of hepatocytes in WD pathology is firmly established. It is less certain whether the excess Cu in hepatocytes is solely responsible for development of WD. To address this issue, we generated a mouse strain for Cre-mediated deletion of Atp7b and inactivated Atp7b selectively in hepatocytes. Atp7bΔ(Hep) mice accumulate copper in the liver, have elevated urinary copper, lack holo-ceruloplasmin, but show no liver disease for up to 30 weeks...
April 20, 2017: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/28427851/circulating-ceruloplasmin-ceruloplasmin-associated-genes-and-the-incidence-of-atrial-fibrillation-in-the-atherosclerosis-risk-in-communities-study
#8
Antonio P Arenas de Larriva, Faye L Norby, Lin Y Chen, Elsayed Z Soliman, Ron C Hoogeveen, Dan E Arking, Laura R Loehr, Alvaro Alonso
BACKGROUND: Ceruloplasmin (CP) may promote structural changes in the atrium making it more arrhythmogenic. We assessed the associations between CP, CP-associated genetic variants, and incident atrial fibrillation (AF) in the Atherosclerosis Risk in Communities (ARIC) study. METHODS AND RESULTS: We studied 10,059 men and women without prevalent AF aged 53 to 75years in 1996-1998 and followed through 2012. Circulating CP was measured in stored blood samples obtained in 1996-1998...
April 6, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28423673/ceruloplasmin-as-a-prognostic-marker-in-patients-with-bile-duct-cancer
#9
In Woong Han, Jin-Young Jang, Wooil Kwon, Taesung Park, Yongkang Kim, Kyoung Bun Lee, Sun-Whe Kim
BACKGROUND AND AIMS: Bile duct cancer is one of the lethal cancers, presenting difficulties in early diagnosis and limited treatment modalities. Despite current advances in biomarker research, most studies have been performed in Western populations. Therefore, the purpose of this study was to determine a prognostic marker for bile duct cancer, especially in Korean patients, whose incidence of bile duct cancer is high. RESULTS: Comparing cancer and normal bile duct tissue, we identified 29091 differentially expressed genes...
March 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/28414228/molecular-aspects-of-a-robust-assay-for-ferroxidase-function-of-ceruloplasmin
#10
Laura Cortes, Blaine R Roberts, Anthony G Wedd, Zhiguang Xiao
Ceruloplasmin (Cp) is one of the most complex multicopper oxidase enzymes and plays an essential role in the metabolism of iron in mammals. Ferrous ion supplied by the ferroportin exporter is converted by Cp to ferric ion that is accepted by plasma metallo-chaperone transferrin. Study of the enzyme at the atomic and molecular level has been hampered by the lack of a suitable ferrous substrate. We have developed the classic chromophoric complex Fe(II)Hx(Tar)2 (H2Tar, 4-(2-thiazolylazo)resorcinol; x = 0-2; overall charge omitted) as a robust substrate for evaluation of the ferroxidase function of Cp and related enzymes...
April 17, 2017: Inorganic Chemistry
https://www.readbyqxmd.com/read/28397223/-analysis-of-clinical-features-and-genetic-mutations-in-a-chinese-family-affected-with-menkes-disease
#11
Xiaorong Shi, Xi Lin, Zhonglin Ke, Shuqing Chen, Bin Wu, Guiling Mo
OBJECTIVE: To delineate the clinical features and potential mutation of the ATP7A gene in a family affected with Menkes disease. METHODS: Clinical data of a patient and his family members were analyzed. Sanger sequencing and multiplex ligation-dependent probe amplification (MLPA) assays were performed to detect the mutation of the ATP7A gene. RESULTS: The patient was admitted at the age of 5 months due to severe epilepsy and marked delayed psychomotor development...
April 10, 2017: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/28397206/relationship-between-maternal-blood-ceruloplasmin-level-catalase-and-myeloperoxidase-activity-and-neural-tube-defects
#12
Orkun Cetin, Erbil Karaman, Baris Boza, Numan Cim, Murat Alisik, Ozcan Erel, Ismet Alkis, Recep Yildizhan, Ali Kolusari, Guler Hanım Sahin
OBJECTIVES: The exact pathogenesis of neural tube defects (NTDs) is poorly understood. We aimed at evaluating maternal anti-oxidant capacity (ceruloplasmin level, myeloperoxidase and catalase activity) in pregnancies complicated by NTDs. MATERIAL AND METHODS: Fifty-four mothers with NTD-affected pregnancies and 61 healthy mothers, matched for gestational age, were recruited. Maternal venous blood samples were obtained after detailed fetal ultrasound examination to measure myeloperoxidase, catalase activity and ceruloplasmin levels...
2017: Ginekologia Polska
https://www.readbyqxmd.com/read/28397151/identification-of-novel-atp7a-mutations-and-prenatal-diagnosis-in-chinese-patients-with-menkes-disease
#13
Binbin Cao, Xiaoping Yang, Yinyin Chen, Qionghui Huang, Ye Wu, Qiang Gu, Jiangxi Xiao, Huixia Yang, Hong Pan, Junya Chen, Yu Sun, Li Ren, Chengfeng Zhao, Yanhua Deng, Yanling Yang, Xingzhi Chang, Zhixian Yang, Yuehua Zhang, Zhengping Niu, Juli Wang, Xiru Wu, Jingmin Wang, Yuwu Jiang
Menkes disease (MD) is a fatal X-linked multisystem disease caused by mutations in ATP7A. In this study, clinical and genetic analysis was performed in 24 male MD patients. Development delay, seizures, kinky coarse hair, and dystonia were found in 24, 22, 24, and 24 patients, respectively. Serum ceruloplasmin/copper tested in 19 patients was low. Abnormal classic features of MD presented in the MRI/MRA of 19 patients. Seventeen mutations of ATP7A were identified in 22 patients. Twelve were novel mutations including three small deletion/insertion, one missense mutation, two nonsense mutations, three splicing-site mutations, and three gross deletions...
April 10, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28392828/screening-of-wilson-s-disease-in-a-psychiatric-population-difficulties-and-pitfalls-a-preliminary-study
#14
Caroline Demily, François Parant, David Cheillan, Emmanuel Broussolle, Alice Pavec, Olivier Guillaud, Lioara Restier, Alain Lachaux, Muriel Bost
BACKGROUND: Wilson's disease (WD) is a rare autosomal-recessive, inherited disorder caused by a mutation in the copper-transporting gene ATP7B affecting the liver and nervous system. About 30% of patients with WD may initially present with psychiatric symptoms, and diagnosis can be difficult to establish. The objectives of the present preliminary study were [1] to evaluate the relevance of serum copper (Cu) and ceruloplasmin (Cp) measures in hospitalized patients with psychiatric disorders; and [2] to identify possible mutations in the ATP7B gene in patients with abnormal biological copper profile...
2017: Annals of General Psychiatry
https://www.readbyqxmd.com/read/28392282/heme-nitrosylated-hemoglobin-and-oxidative-stress-in-women-consuming-combined-contraceptives-clinical-application-of-the-epr-spectroscopy
#15
Irina I Lobysheva, Sandrine van Eeckhoudt, Ahmad Rifahi, Lucie Pothen, Flavia Dei Zotti, Christophe Beauloye, Jean-Luc Balligand
An increased risk of venous thromboembolism was identified in young women consuming combined contraceptive pills (CPs) suggesting a disturbance of vascular homeostasis but the impact of CP on endothelial function and redox status of the vasculature was not thoroughly analyzed. We measured the bioavailability of nitric oxide (NO), a main mediator of vascular homeostasis in a cohort of young female subjects (n = 114) and compared the results in users or not of CPs containing ethinyl estradiol and synthetic progestogens...
April 6, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28383921/icp-ms-ms-based-ionomics-a-validated-methodology-to-investigate-the-biological-variability-of-the-human-ionome
#16
Tobias Konz, Eugenia Migliavacca, Loïc Dayon, Gene L Bowman, Aikaterini Oikonomidi, Julius Popp, Serge Rezzi
We here describe the development, validation and application of a quantitative methodology for the simultaneous determination of 29 elements in human serum using state-of-the-art inductively coupled plasma triple quadrupole mass spectrometry (ICP-MS/MS). This new methodology offers high-throughput elemental profiling using simple dilution of minimal quantity of serum samples. We report the outcomes of the validation procedure including limits of detection/quantification, linearity of calibration curves, precision, recovery and measurement uncertainty...
April 6, 2017: Journal of Proteome Research
https://www.readbyqxmd.com/read/28380026/the-role-of-subcutaneous-adipose-tissue-in-supporting-the-copper-balance-in-rats-with-a-chronic-deficiency-in-holo-ceruloplasmin
#17
Ekaterina Y Ilyechova, Nadezhda V Tsymbalenko, Ludmila V Puchkova
We have previously shown that (1) an acute deficiency in blood serum holo-ceruloplasmin (Cp) developed in rats that were fed fodder containing silver ions (Ag-fodder) for one month and (2) the deficiency in holo-Cp was compensated by non-hepatic holo-Cp synthesis in rats that were chronically fed Ag-fodder for 6 months (Ag-rats). The purpose of the present study is to identify the organ(s) that compensate for the hepatic holo-Cp deficiency in the circulation. This study was performed on rats that were fed Ag-fodder (40 mg Ag·kg-1 body mass daily) for 6 months...
2017: PloS One
https://www.readbyqxmd.com/read/28365121/effects-of-dry-period-length-and-dietary-energy-source-on-inflammatory-biomarkers-and-oxidative-stress-in-dairy-cows
#18
N Mayasari, J Chen, A Ferrari, R M Bruckmaier, B Kemp, H K Parmentier, A T M van Knegsel, E Trevisi
Negative energy balance in dairy cows in early lactation has been associated with increased inflammation and oxidative stress in these cows. The objective of this study was to evaluate the effects of dry period (DP) length and dietary energy source on inflammatory biomarkers and oxidative stress in dairy cows. Holstein-Friesian dairy cows (60 primiparous and 107 multiparous) were assigned randomly to a 3 × 2 factorial design with 3 DP length (0, 30, or 60 d) and 2 early lactation rations (glucogenic or lipogenic)...
March 29, 2017: Journal of Dairy Science
https://www.readbyqxmd.com/read/28360806/possible-effects-of-copper-and-ceruloplasmin-levels-on-auditory-event-potentials-in-boys-with-attention-deficit-hyperactivity-disorder
#19
Özgür Yorbik, Caner Mutlu, Mehmet Fatih Özdağ, Abdullah Olgun, Gül Eryilmaz, Semih Ayta
INTRODUCTION: The aims of the present study were to investigate the relationship between levels of plasma copper (Cu) and ceruloplasmin (Cp) and amplitudes and latencies of P1, N2, and P3 in the parietal and frontal areas of children with attention deficit hyperactivity disorder (ADHD) as well as to compare these Cu levels and event-related potentials (ERPs) indices in controls. METHODS: Boys (n=41) with ADHD were divided into two subgroups according to a median split of plasma Cu and Cp levels, separately...
December 2016: Noro Psikiyatri Arsivi
https://www.readbyqxmd.com/read/28359324/cerebrospinal-fluid-cell-free-mitochondrial-dna-is-associated-with-hiv-replication-iron-transport-and-mild-hiv-associated-neurocognitive-impairment
#20
Sanjay R Mehta, Josué Pérez-Santiago, Todd Hulgan, Tyler R C Day, Jill Barnholtz-Sloan, Haley Gittleman, Scott Letendre, Ronald Ellis, Robert Heaton, Stephanie Patton, Jesse D Suben, Donald Franklin, Debralee Rosario, David B Clifford, Ann C Collier, Christina M Marra, Benjamin B Gelman, Justin McArthur, Allen McCutchan, Susan Morgello, David Simpson, James Connor, Igor Grant, Asha Kallianpur
BACKGROUND: Mitochondria are abundant organelles critical for energy metabolism and brain function. Mitochondrial DNA (mtDNA), released during cellular injury and as part of the innate immune response to viral pathogens, contains CpG motifs that act as TLR-9 ligands. We investigated relationships between cerebrospinal fluid (CSF) cell-free mtDNA levels and HIV viral load (VL), biomarkers of inflammation and iron transport, and neurocognitive (NC) function in the CNS HIV Antiretroviral Therapy Effects Research (CHARTER) cohort...
March 31, 2017: Journal of Neuroinflammation
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