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https://www.readbyqxmd.com/read/28219768/copper-therapy-reduces-intravascular-hemolysis-and-derepresses-ferroportin-in-mice-with-mosaic-mutation-atp7a-mo-ms-an-implication-for-copper-mediated-regulation-of-the-slc40a1-gene-expression
#1
Małgorzata Lenartowicz, Rafał R Starzyński, Aneta Jończy, Robert Staroń, Justyna Antoniuk, Wojciech Krzeptowski, Paweł Grzmil, Aleksandra Bednarz, Olga Pierzchała, Mateusz Ogórek, Zenon Rajfur, Zbigniew Baster, Paweł Lipiński
Mosaic mutant mice displaying functional dysfunction of Atp7a copper transporter (the Menkes ATPase) are an established animal model of Menkes disease and constitute a convenient tool for investigating connections between copper and iron metabolisms. This model allows to explore changes in iron metabolism in suckling mutant mice suffering from systemic copper deficiency as well as in young and adult ones undergone copper therapy, which reduces lethal effect of the Atp7a gene mutation. Our recent study demonstrated that 14-day-old mosaic mutant males display blood cell abnormalities associated with intravascular hemolysis, and show disturbances in the functioning of the hepcidin-ferroportin regulatory axis, which controls systemic iron homeostasis...
February 17, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28164426/mechanisms-of-charge-transfer-in-human-copper-atpases-atp7a-and-atp7b
#2
REVIEW
Francesco Tadini-Buoninsegni, Serena Smeazzetto
ATP7A and ATP7B are Cu(+) -transporting ATPases of subclass IB and play a fundamental role in intracellular copper homeostasis. ATP7A/B transfer Cu(+) ions across the membrane from delivery to acceptor proteins without establishing a free Cu(+) gradient. Transfer of copper across the membrane is coupled to ATP hydrolysis. Current measurements on solid supported membranes (SSM) were performed to investigate the mechanism of copper-related charge transfer across ATP7A and ATP7B. SSM measurements demonstrated that electrogenic copper displacement occurs within ATP7A/B following addition of ATP and formation of the phosphorylated intermediate...
February 5, 2017: IUBMB Life
https://www.readbyqxmd.com/read/28119449/in-vivo-modelling-of-the-pathogenic-effect-of-copper-transporter-mutations-that-cause-menkes-and-wilson-disease-motor-neuropathy-and-susceptibility-to-alzheimer-s-disease
#3
Stephen W Mercer, Jianbin Wang, Richard Burke
Copper is an essential biometal and several inherited diseases are directly associated with a disruption to normal copper homeostasis. The best characterised are the copper deficiency and toxicity disorders Menkes and Wilson disease, caused by mutations in the p-Type Cu-ATPase genes ATP7A and ATP7B respectively. Missense mutations in the C-terminal portion of ATP7A have also been shown to cause Distal Motor Neuropathy while polymorphisms in ATP7B are associated with increased risk of Alzheimer's disease. We have generated a single, in vivo model for studying multiple pathogenic mutations in ATP7 proteins, using Drosophila melanogaster, which has a single orthologue of ATP7A and ATP7B...
January 24, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28115001/host-cell-copper-transporters-ctr1-and-atp7a-are-important-for-influenza-a-virus-replication
#4
Jonathan C Rupp, Manon Locatelli, Alexis Grieser, Andrea Ramos, Patricia J Campbell, Hong Yi, John Steel, Jason L Burkhead, Eric Bortz
BACKGROUND: The essential role of copper in eukaryotic cellular physiology is known, but has not been recognized as important in the context of influenza A virus infection. In this study, we investigated the effect of cellular copper on influenza A virus replication. METHODS: Influenza A/WSN/33 (H1N1) virus growth and macromolecule syntheses were assessed in cultured human lung cells (A549) where the copper concentration of the growth medium was modified, or expression of host genes involved in copper homeostasis was targeted by RNA interference...
January 23, 2017: Virology Journal
https://www.readbyqxmd.com/read/28089327/ceruloplasmin-gene-expression-profile-changes-in-the-rat-mammary-gland-during-pregnancy-lactation-and-involution
#5
Natalia A Platonova, Iurii A Orlov, Sergey A Klotchenko, Victor S Babich, Ekaterina Y Ilyechova, Polina S Babich, Yuri P Garmai, Andrey V Vasin, Nadezhda V Tsymbalenko, Liudmila V Puchkova
Copper metabolism disturbances in mammary gland (MG) cells have severe consequences in newborns. The mechanism that controls the balance of copper in the MG has not been thoroughly characterized. Four primary copper homeostasis genes in mammals: (1) ceruloplasmin (Cp) encoding multifunction multicopper blue (ferr)oxidase; (2) CTR1 encoding high affinity copper importer 1; and (3 and 4) two similar genes encoding Cu(I)/Cu(II)-ATPases P1 type (ATP7A and ATP7B) responsible for copper efflux from the cells and metallation of cuproenzymes formed in the Golgi complex are expressed in MG...
January 3, 2017: Journal of Trace Elements in Medicine and Biology
https://www.readbyqxmd.com/read/28063745/canine-copper-associated-hepatitis
#6
REVIEW
Karen Dirksen, Hille Fieten
Copper-associated hepatitis is recognized with increasing frequency in dogs. The disease is characterized by centrolobular hepatic copper accumulation, leading to hepatitis and eventually cirrhosis. The only way to establish the diagnosis is by histologic assessment of copper distribution and copper quantification in a liver biopsy. Treatment with the copper chelator d-penicillamine is the most commonly used treatment. In addition, a low-copper/high-zinc diet can help prevent accumulation or reaccumulation of hepatic copper...
January 5, 2017: Veterinary Clinics of North America. Small Animal Practice
https://www.readbyqxmd.com/read/27980217/the-association-between-copper-transporters-and-the-prognosis-of-cancer-patients-undergoing-chemotherapy-a-meta-analysis-of-literatures-and-datasets
#7
REVIEW
Si Sun, Jing Cai, Qiang Yang, Simei Zhao, Zehua Wang
Copper transporter 1 (CTR1), copper transporter 2 (CTR2), copper-transporting p-type adenosine triphosphatase 1 and 2 (ATP7A and ATP7B) are key mediators of cellular cisplatin, carboplatin and oxaliplatin accumulation. In this meta-analysis, we aimed to evaluate the relation of CTR1, CTR2, ATP7A and ATP7B to overall survival (OS), progression-free survival (PFS), disease-free survival (DFS) and treatment response (TR) of cancer patients who received chemotherapy based on published literatures, the Gene Expression Omnibus (GEO) and the Cancer Genome Atlas (TCGA) datasets...
December 12, 2016: Oncotarget
https://www.readbyqxmd.com/read/27896900/molecular-features-of-copper-binding-proteins-involved-in-copper-homeostasis
#8
REVIEW
Giuseppe Inesi
Copper has a wide and important role in biological systems, determining conformation and activity of many metalloproteins and enzymes, such as cytochrome oxidase and superoxide dismutase . Furthermore, due to its possible reactivity with nonspecific proteins and toxic effects, elaborate systems of absorption, concentration buffering, delivery to specific protein sites and elimination, require a complex system including small carriers, chaperones and active transporters. The P-type copper ATPases ATP7A and ATP7B provide an important system for acquisition, active transport, distribution and elimination of copper...
November 28, 2016: IUBMB Life
https://www.readbyqxmd.com/read/27881675/the-intestinal-copper-exporter-cua-1-is-required-for-systemic-copper-homeostasis-in-caenorhabditis-elegans
#9
Haarin Chun, Anuj Kumar Sharma, Jaekwon Lee, Jefferson Chan, Shang Jia, Byung-Eun Kim
Copper plays key catalytic and regulatory roles in biochemical processes essential for normal growth, development, and health. Defects in copper metabolism cause Menkes and Wilson's disease, myeloneuropathy, and cardiovascular disease and are associated with other pathophysiological states. Consequently, it is critical to understand the mechanisms by which organisms control the acquisition, distribution, and utilization of copper. The intestinal enterocyte is a key regulatory point for copper absorption into the body; however, the mechanisms by which intestinal cells transport copper to maintain organismal copper homeostasis are poorly understood...
January 6, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27878136/phenotypic-convergence-of-menkes-and-wilson-disease
#10
Boglarka Bansagi, David Lewis-Smith, Endre Pal, Jennifer Duff, Helen Griffin, Angela Pyle, Juliane S Müller, Gabor Rudas, Zsuzsanna Aranyi, Hanns Lochmüller, Patrick F Chinnery, Rita Horvath
Menkes disease is an X-linked multisystem disorder with epilepsy, kinky hair, and neurodegeneration caused by mutations in the copper transporter ATP7A. Other ATP7A mutations have been linked to juvenile occipital horn syndrome and adult-onset hereditary motor neuropathy.(1,2) About 5%-10% of the patients present with "atypical Menkes disease" characterized by longer survival, cerebellar ataxia, and developmental delay.(2) The intracellular copper transport is regulated by 2 P type ATPase copper transporters ATP7A and ATP7B...
December 2016: Neurology. Genetics
https://www.readbyqxmd.com/read/27877224/the-role-of-copper-transporter-atp7a-in-platinum-resistance-of-esophageal-squamous-cell-cancer-escc
#11
Zhuang-Hua Li, Rongjie Zheng, Jing-Tang Chen, Jun Jia, Miaozhen Qiu
Purpose: Platinum derivatives, such as cisplatin (DDP), carboplatin and oxaliplatin, are widely used components of modern cancer chemotherapy including esophageal squamous cell cancer (ESCC). However, their roles are limited by the impact of intrinsic/acquired resistance mechanisms on tumor responses. Recent studies have shown that the mammalian copper transporters CTR1, ATP7A and ATP7B are involved in cisplatin-resistance to some cancers. Methods: The cytotoxicities of DDP in different cell lines were determined using the MTT assay...
2016: Journal of Cancer
https://www.readbyqxmd.com/read/27806319/ammonium-tetrathiomolybdate-treatment-targets-the-copper-transporter-atp7a-and-enhances-sensitivity-of-breast-cancer-to-cisplatin
#12
Cristine L Chisholm, Haitao Wang, Ada Hang-Heng Wong, Guelaguetza Vazquez-Ortiz, Weiping Chen, Xiaoling Xu, Chu-Xia Deng
Cisplatin is an effective breast cancer drug but resistance often develops over prolonged chemotherapy. Therefore, we performed a candidate approach RNAi screen in combination with cisplatin treatment to identify molecular pathways conferring survival advantages. The screen identified ATP7A as a therapeutic target. ATP7A is a copper ATPase transporter responsible for intercellular movement and sequestering of cisplatin. Pharmaceutical replacement for ATP7A by ammonium tetrathiomolybdate (TM) enhanced cisplatin treatment in breast cancer cells...
October 31, 2016: Oncotarget
https://www.readbyqxmd.com/read/27714044/knockdown-of-copper-transporting-atpase-1-atp7a-impairs-iron-flux-in-fully-differentiated-rat-iec-6-and-human-caco-2-intestinal-epithelial-cells
#13
Jung-Heun Ha, Caglar Doguer, James F Collins
Intestinal iron absorption is highly regulated since no mechanism for iron excretion exists. We previously demonstrated that expression of an intestinal copper transporter (Atp7a) increases in parallel with genes encoding iron transporters in the rat duodenal epithelium during iron deprivation (Am. J. Physiol.: Gastrointest. Liver Physiol., 2005, 288, G964-G971). This led us to postulate that Atp7a may influence intestinal iron flux. Therefore, to test the hypothesis that Atp7a is required for optimal iron transport, we silenced Atp7a in rat IEC-6 and human Caco-2 cells...
September 1, 2016: Metallomics: Integrated Biometal Science
https://www.readbyqxmd.com/read/27690074/effects-of-octylphenol-and-bisphenol-a-on-the-metal-cation-transporter-channels-of-mouse-placentas
#14
Jae-Hwan Lee, Changhwan Ahn, Hee Young Kang, Eui-Ju Hong, Sang-Hwan Hyun, Kyung-Chul Choi, Eui-Bae Jeung
Octylphenol (OP) and bisphenol A (BPA) are known as endocrine-disrupting chemicals (EDCs). During pregnancy, the expression of steroid hormone receptors is controlled by maternal and fetal nutrition. To evaluate the impact of EDCs during pregnancy, ethinyl estradiol (EE, 0.2 mg/kg/day), OP (50 mg/kg/day), and BPA (50 mg/kg/day) were administered to pregnant mice. The mRNA levels of TRPV6 (transient receptor potential cation channels in subfamily V, member 6) decreased significantly by EE and OP. The PMCA1 (ATPase, Ca(++) transporting, plasma membrane 1) mRNA and protein levels decreased significantly by EE, OP, and BPA...
September 28, 2016: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/27631241/recurrent-spontaneous-subserosal-hematoma-of-ileum-causing-intestinal-obstruction-in-a-patient-with-menkes-disease-a-case-report
#15
Shu-Chao Weng, Chyong-Hsin Hsu, Nien-Lu Wang, Shuan-Pei Lin, Chuen-Bin Jiang
BACKGROUND: Menkes disease (MD) is a disorder of copper metabolism due to ATP7A gene mutation that leads to severe copper deficiency. Deformed blood vessels can be found in many parts of the body, and intracranial hematoma is generally reported. METHODS: We report a Taiwanese boy with MD who had recurrent spontaneous subserosal hematoma of ileum presenting as intestinal obstruction, with the 2 episodes 23 months apart. The patient returned to the usual physical status after surgical removal of the hematoma...
September 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27629586/diagnostic-copper-imaging-of-menkes-disease-by-synchrotron-radiation-generated-x-ray-fluorescence-analysis
#16
Miyuki Kinebuchi, Akihiro Matsuura, Tohru Kiyono, Yumiko Nomura, Sachiko Kimura
Copper (Cu) is an indispensable metal for normal development and function of humans, especially in central nervous system (CNS). However, its redox activity requires accurate Cu transport system. ATP7A, a main Cu(2+) transporting-ATPase, is necessary to efflux Cu across the plasma membrane and synthesize cuproenzymes. Menkes disease (MD) is caused by mutations in ATP7A gene. Clinically, MD is Cu deficiency syndrome and is treated with Cu-histidine injections soon after definite diagnosis. But outcome of the most remains poor...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27587995/metal-dependent-regulation-of-atp7a-and-atp7b-in-fibroblast-cultures
#17
Malgorzata Lenartowicz, Torben Moos, Mateusz Ogórek, Thomas G Jensen, Lisbeth B Møller
Deficiency of one of the copper transporters ATP7A and ATP7B leads to the rare X-linked disorder Menkes Disease (MD) or the rare autosomal disorder Wilson disease (WD), respectively. In order to investigate whether the ATP7A and the ATP7B genes may be transcriptionally regulated, we measured the expression level of the two genes at various concentrations of iron, copper, and insulin. Treating fibroblasts from controls or from individuals with MD or WD for 3 and 10 days with iron chelators revealed that iron deficiency led to increased transcript levels of both ATP7A and ATP7B...
2016: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/27516958/transcriptome-analysis-of-copper-homeostasis-genes-reveals-coordinated-upregulation-of-slc31a1-sco1-and-cox11-in-colorectal-cancer
#18
Vincenza Barresi, Angela Trovato-Salinaro, Giorgia Spampinato, Nicolò Musso, Sergio Castorina, Enrico Rizzarelli, Daniele Filippo Condorelli
Copper homeostasis and distribution is strictly regulated by a network of transporters and intracellular chaperones encoded by a group of genes collectively known as copper homeostasis genes (CHGs). In this work, analysis of The Cancer Genome Atlas database for somatic point mutations in colorectal cancer revealed that inactivating mutations are absent or extremely rare in CHGs. Using oligonucleotide microarrays, we found a strong increase in mRNA levels of the membrane copper transporter 1 protein [CTR1; encoded by the solute carrier family 31 member 1 gene (SLC31A1 gene)] in our series of colorectal carcinoma samples...
August 2016: FEBS Open Bio
https://www.readbyqxmd.com/read/27509383/effects-of-waterborne-cu-exposure-on-intestinal-copper-transport-and-lipid-metabolism-of-synechogobius-hasta
#19
Feng Chen, Zhi Luo, Guang-Hui Chen, Xi Shi, Xu Liu, Yu-Feng Song, Ya-Xiong Pan
The present study was conducted to explore the effects of waterborne Cu exposure on intestinal Cu transport and lipid metabolism of Synechogobius hasta. S. hasta were exposed to 0, 0.4721 and 0.9442μM Cu, respectively. Sampling occurred on days 0, 21 and 42, respectively. Growth performance, intestinal lipid deposition, Cu content, and activities and mRNA expression of enzymes and genes involved in Cu transport and lipid metabolism were analyzed. Cu exposure decreased WG and SGR on days 21 and 42. Cu exposure increased intestinal Cu and lipid contents...
September 2016: Aquatic Toxicology
https://www.readbyqxmd.com/read/27498968/molecular-phylogenetic-analysis-of-nuclear-genes-suggests-a-cenozoic-over-water-dispersal-origin-for-the-cuban-solenodon
#20
Jun J Sato, Satoshi D Ohdachi, Lazaro M Echenique-Diaz, Rafael Borroto-Páez, Gerardo Begué-Quiala, Jorge L Delgado-Labañino, Jorgelino Gámez-Díez, José Alvarez-Lemus, Son Truong Nguyen, Nobuyuki Yamaguchi, Masaki Kita
The Cuban solenodon (Solenodon cubanus) is one of the most enigmatic mammals and is an extremely rare species with a distribution limited to a small part of the island of Cuba. Despite its rarity, in 2012 seven individuals of S. cubanus were captured and sampled successfully for DNA analysis, providing new insights into the evolutionary origin of this species and into the origins of the Caribbean fauna, which remain controversial. We conducted molecular phylogenetic analyses of five nuclear genes (Apob, Atp7a, Bdnf, Brca1 and Rag1; total, 4,602 bp) from 35 species of the mammalian order Eulipotyphla...
2016: Scientific Reports
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