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copper metabolism

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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/29340742/-wilson-disease
#2
D Huster
Wilson disease is a rare hereditary disorder of copper metabolism. The genetic defect is caused by various mutations in the copper-transporting enzyme ATP7B, located mainly in the liver and brain. Clinical symptoms are highly variable, with any combination of hepatic and/or neurological or psychiatric manifestations. The age of onset varies from early childhood to young adults and can even be manifested in later ages. The clinical diagnosis is based on a combination of clinical, biochemical and molecular markers...
January 16, 2018: Der Internist
https://www.readbyqxmd.com/read/29336469/transcriptional-regulation-of-hiv-1-host-factor-commd1-by-the-sp-family
#3
Eriko Kudo, Manabu Taura, Mary Ann Suico, Hiroki Goto, Hirofumi Kai, Seiji Okada
Copper metabolism Murr1 domain containing 1 (COMMD1) has multiple functions in the regulation of protein stability at the plasma membrane and in the cytoplasm. However, the regulation of COMMD1 transcriptional has remained to be elucidated. In the present study, the 5'‑flanking region (‑1,192/+83 bp) of the human COMMD1 gene was cloned. It was observed that the COMMD1 promoter region contains GC‑rich region that has 7 putative Sp1‑binding sites via in silico analysis. The proximal promoter region at ‑289/+83 bp was required for COMMD1 basal promoter activity by deletion constructs of COMMD1 promoter...
January 15, 2018: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/29334758/anticancer-thiosemicarbazones-chemical-properties-interaction-with-iron-metabolism-and-resistance-development
#4
Petra Heffeter, Veronika F S Pape, Eva A Enyedy, Bernhard K Keppler, Gergely Szakas, Christian R Kowol
SIGNIFICANCE: During the last decades, thiosemicarbazones have been clinically developed for a variety of diseases including tuberculosis, viral infections, malaria and cancer. With regard to malignant diseases, the class of α-N-heterocyclic thiosemicarbazones, and here especially Triapine, was intensively developed in multiple clinical phase I/II trials. Recent Advances: Very recently two new derivatives, namely COTI-2 and DpC have entered phase I evaluation. Based on the strong metal-chelating/metal-interacting properties of thiosemicarbazones, interference with the cellular iron (and copper) homeostasis is assumed to play an important role in their biological activity...
January 15, 2018: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/29334749/-unusual-history-of-wilson-disease-a-case-report-and-review-of-the-literature
#5
František Nehaj, Marianna Kubašková, Michal Mokáň, Juraj Sokol, Vladimír Nosáľ, Kamil Zeleňák, Marián Mokáň
Wilson disease (WD) belongs to autosomal recessive genetic metabolic disorders with gene mutation ATP7B located on 13th chromosome. The enzyme ATPase plays an important role in WD. It facilitates excretion of copper into bile. This gene is responsible for modification of apoceruloplasmin. In this disease, it leads to insufficient release of copper from organism and accumulation of copper in organs such as liver, brain which can cause dysfunction of a certain organ. According to specific symptoms, we can divide WD into psychiatric, neurologic or hepatic form...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29333545/dynamic-changes-in-copper-homeostasis-and-post-transcriptional-regulation-of-atp7a-during-myogenic-differentiation
#6
Katherine E Vest, Amanda L Paskavitz, Joseph B Lee, Teresita Padilla-Benavides
Copper (Cu) is an essential metal required for activity of a number of redox active enzymes that participate in critical cellular pathways such as metabolism and cell signaling. Because it is also a toxic metal, Cu must be tightly controlled by a series of transporters and chaperone proteins that regulate Cu homeostasis. The critical nature of Cu is highlighted by the fact that mutations in Cu homeostasis genes cause pathologic conditions such as Menkes and Wilson diseases. While Cu homeostasis in highly affected tissues like the liver and brain is well understood, no study has probed the role of Cu in development of skeletal muscle, another tissue that often shows pathology in these conditions...
January 15, 2018: Metallomics: Integrated Biometal Science
https://www.readbyqxmd.com/read/29328033/-elemental-status-of-patients-with-various-forms-of-vitiligo
#7
N I Tsiskarishvili, A Katsitadze, N V Tsiskarishvili, I Charischarishvili
Vitiligo is a multifactorial disease in which, in each specific case of its manifestation, different mechanisms of its pathogenesis and different levels of melanin formation in the skin can be involved. Skin is one of the most metabolically active organs. Carrying out a number of vital functions (barrier, protective, respiratory, excretory, metabolic, immune, etc.), it needs microelementss. Of the 92 naturally occurring chemical elements, 81 are found in the human body. Lack of the vital elements, leads to the emergence of diseases, which are based on deficiency, excess or imbalance of micro- and macroelements in the body...
December 2017: Georgian Medical News
https://www.readbyqxmd.com/read/29325617/wilson-disease-and-related-copper-disorders
#8
Matthew T Lorincz
Copper is a required cofactor for enzymes in critical metabolic pathways. Mutations in copper metabolism genes or abnormalities in copper metabolism result in disease from copper excess or deficiency. Wilson disease (WD) is an autosomal-recessive disease caused by mutations in the ATP7B gene which encodes a copper-transporting ATPase. Over 500 different WD mutations throughout the ATP7B gene have been described, most of which are missense mutations. Mutations in both ATP7B alleles result in abnormal copper metabolism and subsequent toxic accumulation of copper...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29323501/the-pivotal-role-of-copper-in-neurodegeneration-a-new-strategy-for-the-therapy-of-neurodegenerative-disorders
#9
Roberta Giampietro, Francesco Spinelli, Marialessandra Contino, Nicola Antonio Colabufo
Copper is an essential trace element for human body since it is a cofactor of several enzymes and proteins and plays a pivotal role in several biological functions (e.g., respiration, protection from oxidative damage, iron metabolism, etc.), also including the central nervous system development and functioning (e.g. synthesis of neurotransmitters, myelination, activation of neuropeptides, etc.). Therefore, copper dysmetabolism is associated with different toxic effects, mainly represented by oxidative stress, and it has been reported in many neurodegenerative disorders, such as Wilson's disease, Menkes disease, Alzheimer's disease, Parkinson's disease, and Amyotrophic Lateral Sclerosis...
January 11, 2018: Molecular Pharmaceutics
https://www.readbyqxmd.com/read/29321352/genetic-analysis-of-55-northern-vietnamese-patients-with-wilson-disease-seven-novel-mutations-in-atp7b
#10
Le Anh Tuan Pham, Trong Tue Nguyen, Hoang Bich Nga Le, Dat Quoc Tran, Cam Tu Ho, Thinh Huy Tran, Van Thanh Ta, The Hung Bui, Van Khanh Tran
Wilson disease (WD) is an autosomal recessive disorder of copper metabolism. The gene responsible for WD was discovered in 1993 and is located on chromosome 13 at 13q14.3. It encodes a copper-specific transporting P-type ATPase. Early diagnosis can improve treatment outcome and decrease the rate of disability or even mortality.We used Sanger sequencing to identify mutation hot spots in 55 northern Vietnamese with a clinical diagnosis of WD. Mutations were screened and detected by direct DNA sequencing. A total of 26 different ATP7B gene mutations were identified, including seven novel mutations (five nonsense and two missense mutations)...
December 2017: Journal of Genetics
https://www.readbyqxmd.com/read/29316463/electrochemical-and-microbial-community-responses-of-electrochemically-active-biofilms-to-copper-ions-in-bioelectrochemical-systems
#11
Yaping Zhang, Guanqun Li, Jing Wen, Yangao Xu, Jian Sun, Xun-An Ning, Xingwen Lu, Yujie Wang, Zuoyi Yang, Yong Yuan
Heavy metals play an important role in the conductivity of solution, power generation and activity of microorganisms in bioelectrochemical systems (BESs). However, effect of heavy metal on the process of exoelectrogenesis metabolism and extracellular electron transfer of electrochemically active biofilms (EABs) was poorly understood. Herein, we investigated the impact of Cu2+ at gradually increasing concentration on the morphological and electrochemical performance and bacterial communities of anodic biofilms in mixed-culture BESs...
January 5, 2018: Chemosphere
https://www.readbyqxmd.com/read/29305509/transcriptional-responses-of-pseudomonas-aeruginosa-to-potable-water-and-freshwater
#12
Erika L English, Kristin C Schutz, Graham G Willsey, Matthew J Wargo
Many Pseudomonas aeruginosa infections are derived from residential, recreational, or surface water sources, thus, these environments represent an important pre-infection niche. To better understand P. aeruginosa biology in these environments, we quantified transcriptional changes by microarray after exposure to diluted LB, diluted R2B, potable tap water, and freshwater from a eutrophic pond. qRT-PCR confirmed conservation of these responses in other water sources, and competition experiments were used to test the importance of three implicated metabolic pathways...
January 5, 2018: Applied and Environmental Microbiology
https://www.readbyqxmd.com/read/29305420/in-vitro-reconstitution-functional-dissection-and-mutational-analysis-of-metal-ion-transport-by-mitoferrin-1
#13
Eric T Christenson, Austin S Gallegos, Anirban Banerjee
Iron is universally important to cellular metabolism and mitoferrins-1 and -2 have been proposed to be the iron importers of mitochondria, the cell's assembly plant of heme and iron-sulfur clusters. These iron-containing prosthetic groups are critical for a host of physiological processes ranging from oxygen transport and energy consumption to maintaining protein structural integrity. Mitoferrin-1 (Mfrn1) belongs to the mitochondrial carrier (MC) family and is atypical given its putative metallic cargo; most MCs transport nucleotides, amino acids, or other small to medium-size metabolites...
January 5, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29305331/differential-expression-of-micrornas-in-hemocytes-from-white-shrimp-litopenaeus-vannamei-under-copper-stress
#14
Hui Guo, Zhi-Cheng Lu, Xiao-Wen Zhu, Chun-Hua Zhu, Cheng-Gui Wang, Yu-Chun Shen, Wei Wang
MicroRNAs (miRNAs) are small noncoding RNAs that regulate diverse cellular processes, including organismal stress response, through posttranscriptional repression of gene transcripts. They are known to have antiviral functions in aquatic crustacean species, but little is known about the role of miRNAs against environmental stress caused by Cu, a common chemical contaminant in aquatic environment. We performed small RNA sequencing to characterize the differentially expressed microRNAs in Cu exposed shrimp. A total of 4524 known miRNAs and 73 novel miRNAs were significantly (P < ...
January 2, 2018: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/29301755/copper-64-labeled-daratumumab-as-a-pet-ct-imaging-tracer-for-multiple-myeloma
#15
Enrico Caserta, Junie Chea, Megan Minnix, Domenico Viola, Steven Vonderfecht, Paul Yazaki, Desiree Crow, Jihane Khalife, James F Sanchez, Joycelynne M Palmer, Susanta Hui, Nadia Carlesso, Jonathan Keats, Young Kim, Ralf Buettner, Guido Marcucci, Steven Rosen, John Shively, David Colcher, Amrita Krishnan, Flavia Pichiorri
As a growing number of patients with multiple myeloma (MM) respond to upfront therapies while eventually relapsing in a time frame that is often non-predictable, attention has increasingly focused on developing novel diagnostic criteria to also account for disease dissemination. Positron emission tomography/computed tomography (PET/CT) is often used as a non-invasive monitoring strategy to assess cancer cell dissemination, but because the uptake of the currently used radiotracer 18fluoro-deoxyglucose (18F-FDG) is a function of the metabolic activity of both malignant and non-malignant cells, results frequently lack sufficient specificity...
January 4, 2018: Blood
https://www.readbyqxmd.com/read/29286645/oxygen-activation-and-radical-transformations-in-heme-proteins-and-metalloporphyrins
#16
Xiongyi Huang, John T Groves
As a result of the adaptation of life to an aerobic environment, nature has evolved a panoply of metalloproteins for oxidative metabolism and protection against reactive oxygen species. Despite the diverse structures and functions of these proteins, they share common mechanistic grounds. An open-shell transition metal like iron or copper is employed to interact with O2 and its derived intermediates such as hydrogen peroxide to afford a variety of metal-oxygen intermediates. These reactive intermediates, including metal-superoxo, -(hydro)peroxo, and high-valent metal-oxo species, are the basis for the various biological functions of O2-utilizing metalloproteins...
December 29, 2017: Chemical Reviews
https://www.readbyqxmd.com/read/29278844/the-effects-of-bio-available-copper-on-macrolide-antibiotic-resistance-genes-and-mobile-elements-during-tylosin-fermentation-dregs-co-composting
#17
Bo Zhang, Meng Meng Wang, Bing Wang, Yanjun Xin, Jiaqi Gao, Huiling Liu
In this study, aerobic co-composting of tylosin fermentation dregs (TFDs) and sewage sludge with different adding concentrations of copper (Cu) was investigated to inspect the fate of antibiotic resistance genes (ARGs), metal resistance genes (MRGs) and mobile genetic elements (MGEs). Results showed that two concentrations of Cu did affect not only the abiotic factors but the relative abundances of resistance genes. High concentration of Cu inhibited the metabolic capacity of microbial community and the nitrogen-fixing process while had little effect on the degradation of TYL and TOC...
December 18, 2017: Bioresource Technology
https://www.readbyqxmd.com/read/29277024/copper-redox-chemistry-of-plant-frataxins
#18
Manu Sánchez, Òscar Palacios, Celeste Buchensky, Laura Sabio, Diego Fabian Gomez-Casati, Maria Ayelen Pagani, Mercè Capdevila, Silvia Atrian, Jose M Dominguez-Vera
The presence of a conserved cysteine residue in the C-terminal amino acid sequences of plant frataxins differentiates these frataxins from those of other kingdoms and may be key in frataxin assembly and function. We report a full study on the ability of Arabidopsis (AtFH) and Zea mays (ZmFH-1 and ZmFH-2) frataxins to assemble into disulfide-bridged dimers by copper-driven oxidation and to revert to monomers by chemical reduction. We monitored the redox assembly-disassembly process by electrospray ionization mass spectrometry, electrophoresis, UV-Vis spectroscopy, and fluorescence measurements...
November 27, 2017: Journal of Inorganic Biochemistry
https://www.readbyqxmd.com/read/29274988/anaerobic-biodegradation-of-biofuels-and-their-impact-on-the-corrosion-of-a-cu-ni-alloy-in-marine-environments
#19
Renxing Liang, Egemen Aydin, Sylvie Le Borgne, Jan Sunner, Kathleen E Duncan, Joseph M Suflita
Fuel biodegradation linked to sulfate reduction can lead to corrosion of the metallic infrastructure in a variety of marine environments. However, the biological stability of emerging biofuels and their potential impact on copper-nickel alloys commonly used in marine systems has not been well documented. Two potential naval biofuels (Camelina-JP5 and Fisher-Tropsch-F76) and their petroleum-derived counterparts (JP5 and F76) were critically assessed in seawater/sediment incubations containing a metal coupon (70/30 Cu-Ni alloy)...
December 13, 2017: Chemosphere
https://www.readbyqxmd.com/read/29259852/epidemiology-diagnosis-and-treatment-of-wilson-s-disease
#20
REVIEW
Jing Liu, Jing Luan, Xiaoyan Zhou, Yazhou Cui, Jinxiang Han
Wilson's disease (WD) is an autosomal recessive disease caused by a mutation of the ATP7B gene, resulting in abnormal copper metabolism. The major clinical features of WD include liver disease, neurological disorders, K-F rings, and osteoporosis. The prevalence of WD in China is higher than that in Western countries. Early diagnosis and lifelong treatment will lead to better outcomes. Drugs such as sodium dimercaptosuccinate (Na-DMPS), Zn, and Gandou Decoction can be used to treat WD. Some studies have shown that the combination of traditional Chinese medicine and Western medicine is the best approach to treating WD...
November 2017: Intractable & Rare Diseases Research
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