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copper metabolism

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https://www.readbyqxmd.com/read/28442376/copper-sulfate-pretreatment-prevents-mitochondrial-electron-transport-chain-damage-and-apoptosis-against-mpp-induced-neurotoxicity
#1
Moisés Rubio-Osornio, Marisol Orozco-Ibarra, Araceli Díaz-Ruiz, Eduardo Brambila, Marie-Catherine Boll, Antonio Monroy-Noyola, Jorge Guevara, Sergio Montes, Camilo Ríos
Intrastriatal injection of 1-methyl-4-phenylpyridinium (MPP(+)) is considered a model to reproduce some biochemical alterations observed in Parkinson's disease (PD) patients. Among those alterations, inhibition of mitochondrial complex I activity, increased free radical production and reduced antioxidant responses have been reported. Copper (Cu) plays an important role in the metabolism and antioxidative responses through its participation as a cofactor in the cytochrome c oxidase enzyme (COX), Cu/Zn-superoxide dismutase (Cu/Zn-SOD), and metallothioneins...
April 22, 2017: Chemico-biological Interactions
https://www.readbyqxmd.com/read/28433110/animal-models-of-wilson-disease
#2
Valentina Medici, Dominik Huster
Wilson disease (WD) is caused by ATPase copper-transporting beta (ATP7B) mutations and results in copper toxicity in liver and brain. Although the defective gene was identified in 1993, the specific mechanisms underlying copper toxicity and the remarkable phenotypic diversity of the disease are still poorly understood. Animal models harboring defects in the ATP7B homolog have helped to reveal new insights into pathomechanisms of WD. Four rodent models with ATP7B gene defects have been described - the Long-Evans Cinnamon (LEC) rat, inbred mouse models (toxic milk (tx), the Jackson Laboratory toxic milk (tx-j)), and the genetically engineered ATP7B(-/-) (knockout) mouse - all of which develop liver disease to different extents...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28433108/genetic-and-environmental-modifiers-of-wilson-disease
#3
Valentina Medici, Karl-Heinz Weiss
Wilson disease (WD) is characterized by remarkable variety in its phenotypic presentation. Patients with WD can present with hepatic, neurologic, and psychiatric symptoms combined in different and unpredictable ways. Importantly, no convincing phenotype-genotype correlation has ever been identified, opening the possibility that other genes, aside from ATPase copper-transporting beta (ATP7B), are involved in the pathogenesis of this condition. In addition, modifier genes, or genes that can affect the expression of other genes, may be involved...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28433101/wilson-disease-currently-used-anticopper-therapy
#4
Anna Członkowska, Tomasz Litwin
Wilson disease (WD) is a genetic disorder of copper metabolism that can be treated successfully with pharmacologic treatment. Two groups of drugs are currently used: chelators (e.g., d-penicillamine and trientine), which increase urinary copper excretion, and zinc salts, which inhibit copper absorption in the digestive tract. The mechanisms of action lead to a negative copper balance, stopping pathologic accumulation of copper in the tissues and clearing affected organs of copper overload. Due to a lack of prospective clinical trials, the use of drugs depends mainly on center experience and the accessibility in different countries or regions...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28433099/other-organ-involvement-and-clinical-aspects-of-wilson-disease
#5
Karolina Dzieżyc, Tomasz Litwin, Anna Członkowska
Wilson disease (WD) is a rare disorder of copper metabolism that presents mainly with hepatic and neuropsychiatric features. Copper accumulates not only in the liver and brain, but also in other organs. Liver injury can also be the cause of secondary impairment of other tissues. Therefore, the clinical manifestation of WD may be renal, cardiac, skin, osteoarticular, or endocrinologic and include other organ disturbances. Renal abnormalities include tubular dysfunction (e.g., renal tubular acidosis, aminoaciduria) and nephrolithiasis...
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
#6
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/28428045/sco2-deficient-mice-develop-increased-adiposity-and-insulin-resistance
#7
Shauna Hill, Sathyaseelan S Deepa, Kavithalakshmi Sataranatarajan, Pavithra Premkumar, Daniel Pulliam, Yuhong Liu, Vanessa Y Soto, Kathleen E Fischer, Holly Van Remmen
Cytochrome c oxidase (COX) is an essential transmembrane protein complex (Complex IV) in the mitochondrial respiratory electron chain. Mutations in genes responsible for the assembly of COX are associated with Leigh syndrome, cardiomyopathy, spinal muscular atrophy and other fatal metabolic disorders in humans. Previous studies have shown that mice lacking the COX assembly protein Surf1 (Surf1(-/-) mice) paradoxically show a number of beneficial metabolic phenotypes including increased insulin sensitivity, upregulation of mitochondrial biogenesis, induction of stress response pathways and increased lifespan...
April 17, 2017: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/28426192/profiling-cellular-substrates-of-lysine-acetyltransferases-gcn5-and-p300-with-orthogonal-labeling-and-click-chemistry
#8
Zhen Han, Chau-Wen Chou, Xiangkun Yang, Michael G Bartlett, Y George Zheng
p300 and GCN5 are two representative histone/lysine acetyltransferase (HAT/KAT) enzymes in mammalian cells. It was recently reported that they possess multiple acyltransferase activities including acetylation, propionylation, and butyrylation of the -amino group of lysine residues of histones and non-histone protein substrates. Although thousands of acetylated substrates and acetylation sites have been identified by mass spectrometry-based proteomic screening, our knowledge about protein acylation, especially the causative connection between individual KAT members and their substrates remain limited...
April 20, 2017: ACS Chemical Biology
https://www.readbyqxmd.com/read/28422987/systematic-review-and-meta-analysis-shows-a-specific-micronutrient-profile-in-people-with-down-syndrome-lower-blood-calcium-selenium-and-zinc-higher-red-blood-cell-copper-and-zinc-and-higher-salivary-calcium-and-sodium
#9
REVIEW
Amene Saghazadeh, Maryam Mahmoudi, Atefeh Dehghani Ashkezari, Nooshin Oliaie Rezaie, Nima Rezaei
Different metabolic profiles as well as comorbidities are common in people with Down Syndrome (DS). Therefore it is relevant to know whether micronutrient levels in people with DS are also different. This systematic review was designed to review the literature on micronutrient levels in people with DS compared to age and sex-matched controls without DS. We identified sixty nine studies from January 1967 to April 2016 through main electronic medical databases PubMed, Scopus, and Web of knowledge. We carried out meta-analysis of the data on four essential trace elements (Cu, Fe, Se, and Zn), six minerals (Ca, Cl, K, Mg, Na, and P), and five vitamins (vitamin A, B9, B12, D, and E)...
2017: PloS One
https://www.readbyqxmd.com/read/28413194/dual-role-of-cellular-prion-protein-in-normal-host-and-alzheimer-s-disease
#10
REVIEW
Takashi Onodera
Using PrP(C)-knockout cell lines, it has been shown that the inhibition of apoptosis through STI1 is mediated by PrP(C)-dependent SOD activation. Antioxidant PrP(C) may contribute to suppression of inflammasome activation. PrP(C) is functionally involved in copper metabolism, signal transduction, neuroprotection, and cell maturation. Recently several reports have shown that PrP(C) participates in trans-membrane signaling processes associated with hematopoietic stem cell replication and neuronal differentiation...
2017: Proceedings of the Japan Academy. Series B, Physical and Biological Sciences
https://www.readbyqxmd.com/read/28409271/unique-presentation-of-cutis-laxa-with-leigh-like-syndrome-due-to-echs1-deficiency
#11
S Balasubramaniam, L G Riley, D Bratkovic, D Ketteridge, N Manton, M J Cowley, V Gayevskiy, T Roscioli, M Mohamed, T Gardeitchik, E Morava, J Christodoulou
Clinical finding of cutis laxa, characterized by wrinkled, redundant, sagging, nonelastic skin, is of growing significance due to its occurrence in several different inborn errors of metabolism (IEM). Metabolic cutis laxa results from Menkes syndrome, caused by a defect in the ATPase copper transporting alpha (ATP7A) gene; congenital disorders of glycosylation due to mutations in subunit 7 of the component of oligomeric Golgi (COG7)-congenital disorders of glycosylation (CDG) complex; combined disorder of N- and O-linked glycosylation, due to mutations in ATPase H+ transporting V0 subunit a2 (ATP6VOA2) gene; pyrroline-5-carboxylate reductase 1 deficiency; pyrroline-5-carboxylate synthase deficiency; macrocephaly, alopecia, cutis laxa, and scoliosis (MACS) syndrome, due to Ras and Rab interactor 2 (RIN2) mutations; transaldolase deficiency caused by mutations in the transaldolase 1 (TALDO1) gene; Gerodermia osteodysplastica due to mutations in the golgin, RAB6-interacting (GORAB or SCYL1BP1) gene; and mitogen-activated pathway (MAP) kinase defects, caused by mutations in several genes [protein tyrosine phosphatase, non-receptor-type 11 (PTPN11), RAF, NF, HRas proto-oncogene, GTPase (HRAS), B-Raf proto-oncogene, serine/threonine kinase (BRAF), MEK1/2, KRAS proto-oncogene, GTPase (KRAS), SOS Ras/Rho guanine nucleotide exchange factor 2 (SOS2), leucine rich repeat scaffold protein (SHOC2), NRAS proto-oncogene, GTPase (NRAS), and Raf-1 proto-oncogene, serine/threonine kinase (RAF1)], which regulate the Ras-MAPK cascade...
April 13, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28408824/cuprous-oxide-nanoparticles-inhibit-prostate-cancer-by-attenuating-the-stemness-of-cancer-cells-via-inhibition-of-the-wnt-signaling-pathway
#12
Ye Wang, Qi-Wei Yang, Qing Yang, Tie Zhou, Min-Feng Shi, Chen-Xia Sun, Xiu-Xia Gao, Yan-Qiong Cheng, Xin-Gang Cui, Ying-Hao Sun
Disordered copper metabolism plays a critical role in the development of various cancers. As a nanomedicine containing copper, cuprous oxide nanoparticles (CONPs) exert ideal antitumor pharmacological effects in vitro and in vivo. Prostate cancer is a frequently diagnosed male malignancy prone to relapse, and castration resistance is the main reason for endocrine therapy failure. However, whether CONPs have the potential to treat castration-resistant prostate cancer is still unknown. Here, using the castration-resistant PC-3 human prostate cancer cell line as a model, we report that CONPs can selectively induce apoptosis and inhibit the proliferation of cancer cells in vitro and in vivo without affecting normal prostate epithelial cells...
2017: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/28400155/histaminergic-gene-polymorphisms-associated-with-sedation-in-clozapine-treated-patients
#13
Anssi Solismaa, Olli Kampman, Leo-Pekka Lyytikäinen, Niko Seppälä, Merja Viikki, Nina Mononen, Terho Lehtimäki, Esa Leinonen
Sedation is a common adverse effect of clozapine treatment, which may be partly related to clozapine binding to histamine receptors in the central nervous system. The objective of this study was to investigate whether single nucleotide polymorphisms (SNPs) in the histaminergic system are associated with sedation in clozapine-treated patients. The study population comprised 237 clozapine-treated, Finnish, Caucasian patients that were diagnosed with schizophrenia and 176 were genotyped using Illumina HumanCoreExome-12 BeadChip...
April 8, 2017: European Neuropsychopharmacology: the Journal of the European College of Neuropsychopharmacology
https://www.readbyqxmd.com/read/28392254/american-society-for-metabolic-and-bariatric-surgery-integrated-health-nutritional-guidelines-for-the-surgical-weight-loss-patient-2016-update-micronutrients
#14
Julie Parrott, Laura Frank, Rebecca Rabena, Lillian Craggs-Dino, Kellene A Isom, Laura Greiman
BACKGROUND: Optimizing postoperative patient outcomes and nutritional status begins preoperatively. Patients should be educated before and after weight loss surgery (WLS) on the expected nutrient deficiencies associated with alterations in physiology. Although surgery can exacerbate preexisting nutrient deficiencies, preoperative screening for vitamin deficiencies has not been the norm in the majority of WLS practices. Screening is important because it is common for patients who present for WLS to have at least 1 vitamin or mineral deficiency preoperatively...
January 19, 2017: Surgery for Obesity and related Diseases: Official Journal of the American Society for Bariatric Surgery
https://www.readbyqxmd.com/read/28389407/d-penicillamine-combined-with-inhibitors-of-hydroperoxide-metabolism-enhances-lung-and-breast-cancer-cell-responses-to-radiation-and-carboplatin-via-h2o2-mediated-oxidative-stress
#15
Sebastian J Sciegienka, Shane R Solst, Kelly C Falls, Joshua D Schoenfeld, Adrienne R Klinger, Natalie L Ross, Samuel N Rodman, Douglas R Spitz, Melissa A Fath
D-penicillamine (DPEN), a copper chelator, has been used in the treatment of Wilson's disease, cystinuria, and rheumatoid arthritis. Recent evidence suggests that DPEN in combination with biologically relevant copper (Cu) concentrations generates H2O2 in cancer cell cultures, but the effects of this on cancer cell responses to ionizing radiation and chemotherapy are unknown. Increased steady-state levels of H2O2 were detected in MB231 breast and H1299 lung cancer cells following treatment with DPEN (100µM) and copper sulfate (15µM)...
April 5, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28388654/triplin-a-small-molecule-reveals-copper-ion-transport-in-ethylene-signaling-from-atx1-to-ran1
#16
Wenbo Li, Randy F Lacey, Yajin Ye, Juan Lu, Kuo-Chen Yeh, Youli Xiao, Laigeng Li, Chi-Kuang Wen, Brad M Binder, Yang Zhao
Copper ions play an important role in ethylene receptor biogenesis and proper function. The copper transporter RESPONSIVE-TO-ANTAGONIST1 (RAN1) is essential for copper ion transport in Arabidopsis thaliana. However it is still unclear how copper ions are delivered to RAN1 and how copper ions affect ethylene receptors. There is not a specific copper chelator which could be used to explore these questions. Here, by chemical genetics, we identified a novel small molecule, triplin, which could cause a triple response phenotype on dark-grown Arabidopsis seedlings through ethylene signaling pathway...
April 2017: PLoS Genetics
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/28376412/effect-of-exogenously-applied-molybdenum-on-its-absorption-and-nitrate-metabolism-in-strawberry-seedlings
#18
Li Liu, Wei Xiao, Ling Li, Dong-Mei Li, Dong-Sheng Gao, Cui-Ying Zhu, Xi-Ling Fu
Molybdenum (Mo)-an essential element of plants-is involved in nitrogen (N) metabolism. Plants tend to accumulate more nitrate and show lower nitrogen use efficiency (NUE) under Mo-deficient conditions. Improving NUE in fruits reduces the negative effect of large applications of chemical fertilizer, but the mechanisms underlying how Mo enhances NUE remain unclear. We cultivated strawberry seedlings sprayed with 0, 67.5, 135, 168.75, or 202.5 g Mo·ha(-1) in a non-soil culture system. The Mo concentration in every plant tissue analyzed increased gradually as Mo application level rose...
March 23, 2017: Plant Physiology and Biochemistry: PPB
https://www.readbyqxmd.com/read/28371651/metals-and-minerals-as-a-biotechnology-feedstock-engineering-biomining-microbiology-for-bioenergy-applications
#19
REVIEW
Indrani Banerjee, Brittany Burrell, Cara Reed, Alan C West, Scott Banta
Developing new feedstocks for the efficient production of biochemicals and biofuels will be a critical challenge as we diversify away from petrochemicals. One possible opportunity is the utilization of sulfide-based minerals in the Earth's crust. Non-photosynthetic chemolithoautotrophic bacteria are starting to be developed to produce biochemicals from CO2 using energy obtained from the oxidation of inorganic feedstocks. Biomining of metals like gold and copper already exploit the native metabolism of these bacteria and these represent perhaps the largest-scale bioprocesses ever developed...
March 31, 2017: Current Opinion in Biotechnology
https://www.readbyqxmd.com/read/28370567/vertically-oriented-and-shape-tailored-electrocatalytic-metal-nanowire-arrays-for-enzyme-free-galactosemia-rapid-diagnosis
#20
Laura García-Carmona, María Cristina González, Alberto Escarpa
Metallic catalytic nanowires such as nickel and copper nanowires (NWs) for electrochemical detection of carbohydrates involved in metabolic rare diseases are proposed. NWs were electrodeposited using a polycarbonate membrane template, which was cut with the desired shape, stuck in double-sided adhesive tape, pasted into a non-conductive substrate and in situ removed. This simple and versatile approach allowed to obtain NWs vertically oriented (v-NWs), which are contained in the double-sided adhesive tape, becoming highly versatile...
April 3, 2017: Chemistry: a European Journal
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