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Copper deficiency

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https://www.readbyqxmd.com/read/28428045/sco2-deficient-mice-develop-increased-adiposity-and-insulin-resistance
#1
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/28409271/unique-presentation-of-cutis-laxa-with-leigh-like-syndrome-due-to-echs1-deficiency
#2
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/28392254/american-society-for-metabolic-and-bariatric-surgery-integrated-health-nutritional-guidelines-for-the-surgical-weight-loss-patient-2016-update-micronutrients
#3
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/28390585/biomarkers-of-oxidative-stress-and-antioxidants-in-severe-asthma-a-prospective-case-control-study
#4
Abigail Bishopp, Ramamurthy Sathyamurthy, Sarah Manney, Craig Webbster, Mamidipudi Thirumala Krishna, Adel H Mansur
BACKGROUND: Bronchial airway inflammation is the hallmark of asthma, which may be driven by an imbalance between oxidative stress and antioxidant defenses. Antioxidants deficiency may play a role, but this has remained unconfirmed. OBJECTIVE: To evaluate the oxidative stress burden and antioxidants defenses in patients with increasing asthma severity. METHODS: This prospective case-control study compared fractional exhaled nitric oxide (FeNO), exhaled breath condensate nitrite/nitrate (EBC-NOx), spirometry, and serum vitamins and trace elements among patients with and without asthma...
April 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/28388674/variation-in-the-mineral-element-concentration-of-moringa-oleifera-lam-and-m-stenopetala-bak-f-cuf-role-in-human-nutrition
#5
Diriba B Kumssa, Edward Jm Joy, Scott D Young, David W Odee, E Louise Ander, Martin R Broadley
BACKGROUND: Moringa oleifera (MO) and M. stenopetala (MS) (family Moringaceae; order Brassicales) are multipurpose tree/shrub species. They thrive under marginal environmental conditions and produce nutritious edible parts. The aim of this study was to determine the mineral composition of different parts of MO and MS growing in their natural environments and their potential role in alleviating human mineral micronutrient deficiencies (MND) in sub-Saharan Africa. METHODS: Edible parts of MO (n = 146) and MS (n = 50), co-occurring cereals/vegetables and soils (n = 95) underneath their canopy were sampled from localities in southern Ethiopia and Kenya...
2017: PloS One
https://www.readbyqxmd.com/read/28386624/mutated-pet117-causes-complex-iv-deficiency-and-is-associated-with-neurodevelopmental-regression-and-medulla-oblongata-lesions
#6
G H Renkema, G Visser, F Baertling, L T Wintjes, V M Wolters, J van Montfrans, G A P de Kort, P G J Nikkels, P M van Hasselt, S N van der Crabben, R J T Rodenburg
The genetic basis of the many progressive, multi systemic, mitochondrial diseases that cause a lack of cellular ATP production is heterogeneous, with defects found both in the mitochondrial genome as well as in the nuclear genome. Many different mutations have been found in the genes encoding subunits of the enzyme complexes of the oxidative phosphorylation system. In addition, mutations in genes encoding proteins involved in the assembly of these complexes are known to cause mitochondrial disorders. Here we describe two sisters with a mitochondrial disease characterized by lesions in the medulla oblongata, as demonstrated by brain magnetic resonance imaging, and an isolated complex IV deficiency and reduced levels of individual complex IV subunits...
April 6, 2017: Human 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
#7
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
#8
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/28362575/review-of-copper-provision-in-the-parenteral-nutrition-of-adults-formula-see-text
#9
Callum Livingstone
The essential trace element copper (Cu) is required for a range of physiologic processes, including wound healing and functioning of the immune system. The correct amount of Cu must be provided in parenteral nutrition (PN) if deficiency and toxicity are to be avoided. While provision in line with the standard recommendations should suffice for most patients, Cu requirements may be higher in patients with increased gastrointestinal losses and severe burns and lower in those with cholestasis. The tests of Cu status that are currently available for clinical use are unreliable...
April 2017: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/28358335/copper-to-zinc-ratio-as-disease-biomarker-in-neonates-with-early-onset-congenital-infections
#10
Monika Wisniewska, Malte Cremer, Lennart Wiehe, Niels-Peter Becker, Eddy Rijntjes, Janine Martitz, Kostja Renko, Christoph Bührer, Lutz Schomburg
Copper (Cu) and zinc (Zn) are essential trace elements for regular development. Acute infections alter their metabolism, while deficiencies increase infection risks. A prospective observational case-control study was conducted with infected (n = 21) and control (n = 23) term and preterm newborns. We analyzed trace element concentrations by X-ray fluorescence, and ceruloplasmin (CP) by Western blot. Median concentration of Cu at birth (day 1) was 522.8 [387.1-679.7] μg/L, and Zn was 1642.4 ± 438.1 μg/L. Cu and Zn correlated positively with gestational age in control newborns...
March 30, 2017: Nutrients
https://www.readbyqxmd.com/read/28356913/posterior-cord-syndrome-and-trace-elements-deficiency-as-an-uncommon-presentation-of-common-variable-immunodeficiency
#11
Yuri Silva Macedo, Ananda Dos Santos Mota, Priscila Morais Monteiro, Angela Cristina Gouvêa Carvalho, Barbara Fernandes Diniz, Pedro Gemal Lanzieri, Ricardo Carneiro Ramos, Luis Otavio Mocarzel, Ronaldo Altenburg Gismondi
Diarrhea is one of the most common symptoms in common variable immunodeficiency, but neurologic manifestations are rare. We presented a 50-year-old woman with recurrent diarrhea and severe weight loss that developed a posterior cord syndrome. Endoscopy found a duodenal villous blunting, intraepithelial lymphocytosis, and lack of plasma cells and magnetic resonance imaging of the spine was normal. Laboratory assays confirmed common variable immunodeficiency syndrome and showed low levels of trace elements (copper and zinc)...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28350115/transition-and-heavy-metals-compared-to-oxidative-parameter-balance-in-patients-with-deep-vein-thrombosis-a-case-control-study
#12
Margherita Ferrante, Maria Fiore, Gea Oliveri Conti, Valerio Fiore, Alfina Grasso, Chiara Copat, Salvatore Santo Signorelli
Our study compared copper (Cu), zinc (Zn) and manganese (Mn), crucial for human normal physio-logy maintenance, and lead (Pb) levels as environmental pollutant, in subjects suffering of deep vein thrombosis of lower limbs (DVTs) vs. healthy subjects. Furthermore, we evaluated oxidative stress parameters, the thiobarbituric acid reactive substances (TBARS) as the sum of malondialdehyde or 1,1,3,3-tetraethoxypropane (MDA) and 4-hydroxynonenal 4-HNE) and cytosolic superoxide dismutase (Cu/Zn-SOD) concentrations in both groups...
March 24, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28347454/minerals-in-thalassaemia-major-patients-an-overview
#13
REVIEW
Zeynep Ozturk, Gizem Esra Genc, Saadet Gumuslu
Thalassaemia major (TM) is a hereditary blood disease characterised by reduced or absent production of beta globin chains. Erythrocyte transfusions are given to raise the haemoglobin level in patients with thalassaemia major. However, transfusions have been related to increased risk of iron overload and tissue damage related to excess iron. Both elevated oxidative stress due to iron overload and increased hemolysis lead to over utilisation of minerals required for antioxidant enzymes activities. Iron chelators have been used to prevent iron overload in thalassaemia major patients, but these chelators have the possibility of removing minerals from the body...
May 2017: Journal of Trace Elements in Medicine and Biology
https://www.readbyqxmd.com/read/28345660/zinc-deficiency-and-low-enterocyte-zinc-transporter-expression-in-human-patients-with-autism-related-mutations-in-shank3
#14
Stefanie Pfaender, Ann Katrin Sauer, Simone Hagmeyer, Katharina Mangus, Leonhard Linta, Stefan Liebau, Juergen Bockmann, Guillaume Huguet, Thomas Bourgeron, Tobias M Boeckers, Andreas M Grabrucker
Phelan McDermid Syndrome (PMDS) is a genetic disorder characterized by features of Autism spectrum disorders. Similar to reports of Zn deficiency in autistic children, we have previously reported high incidence of Zn deficiency in PMDS. However, the underlying mechanisms are currently not well understood. Here, using inductively coupled plasma mass-spectrometry to measure the concentration of Zinc (Zn) and Copper (Cu) in hair samples from individuals with PMDS with 22q13.3 deletion including SHANK3 (SH3 and multiple ankyrin repeat domains 3), we report a high rate of abnormally low Zn/Cu ratios...
March 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28330871/human-mitochondrial-cytochrome-c-oxidase-assembly-factor-cox18-acts-transiently-as-a-membrane-insertase-within-the-subunit-2-maturation-module
#15
Myriam Bourens, Antoni Barrientos
Defects in mitochondrial cytochrome c oxidase or respiratory chain complex IV (CIV) assembly are a frequent cause of human mitochondrial disorders. Specifically, mutations in four conserved assembly factors impinging the biogenesis of the mitochondrion-encoded catalytic core subunit 2 (COX2) result in myopathies. These factors afford stability of newly-synthesized COX2 (the dystonia-ataxia syndrome protein COX20), a protein with two transmembrane domains, and maturation of its copper center CuA (cardiomyopathy proteins SCO1, SCO2 and COA6)...
March 22, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28328213/reversible-coordination-of-boron-aluminum-zinc-magnesium-and-calcium-hydrogen-bonds-to-bent-cul2-fragments-heavy-%C3%AF-complexes-of-the-lightest-coinage-metal
#16
Alexandra Hicken, Andrew J P White, Mark R Crimmin
A series of copper(I) complexes bearing electron-deficient β-diketiminate ligands have been prepared. The study includes [{{ArNC(CR3)}2CH}Cu(η(2)-toluene)n] (Ar = Mes, R = F, n = 0.5, [12·tol]; Ar = C6F5, R = Me, n = 1, [2·tol]; Ar = 2,6-Cl2C6H3, R = H, n = 0.5, [32·tol]). Reactions of [1-3n·tol] with boranes, alanes, a zinc hydride, a magnesium hydride, and a calcium hydride generate the corresponding σ complexes ([1-3·B], [3·B'], [3·Al], [3·Al'], [1-3·Zn], [1·Mg], and [1·Ca]). These species all form reversibly, being in equilibrium with the arene solvates in solution...
March 22, 2017: Inorganic Chemistry
https://www.readbyqxmd.com/read/28321633/dietary-intake-of-minerals-vitamins-and-trace-elements-among-geriatric-population-in-india
#17
Aakriti Gupta, Preetika Khenduja, Ravindra Mohan Pandey, Hem Chandra Sati, Nighat Yaseen Sofi, Umesh Kapil
The geriatric population is at a high risk of developing deficiencies of essential micronutrients such as minerals, vitamins, and trace elements and their related deficiency signs and symptoms. Scarce data is available on the dietary intake of essential micronutrients among geriatric subjects in India. Hence, to fill the gap in the existing knowledge, a community-based cross-sectional study was conducted during 2015-2016 in District Nainital, Uttarakhand State, India. A total of 255 geriatric subjects were enrolled from 30 clusters (villages) identified by using population proportionate to size sampling methodology...
March 20, 2017: Biological Trace Element Research
https://www.readbyqxmd.com/read/28318055/menkes-disease-mimicking-child-abuse
#18
Rebecca J Droms, Jillian F Rork, Riley McLean, Madelena Martin, Leah Belazarian, Karen Wiss
Althouygh Menkes disease has well-recognized neurologic, developmental, and cutaneous features, the initial presentation may resemble child abuse. We describe a 5-month-old boy with multiple fractures indicative of nonaccidental trauma who was ultimately diagnosed with Menkes disease. Copper deficiency leads to connective tissue abnormalities and may result in subdural hematomas, wormian bones, cervical spine defects, rib fractures, and spurring of the long bone metaphyses. Several of these findings, including fractures and subdural hematomas, may be misinterpreted as child abuse...
March 20, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28303526/a-herbivore-tag-and-trace-system-reveals-contact-and-density-dependent-repellence-of-a-root-toxin
#19
Zoe Bont, Carla Arce, Meret Huber, Wei Huang, Adrien Mestrot, Craig J Sturrock, Matthias Erb
Foraging behavior of root feeding organisms strongly affects plant-environment-interactions and ecosystem processes. However, the impact of plant chemistry on root herbivore movement in the soil is poorly understood. Here, we apply a simple technique to trace the movement of soil-dwelling insects in their habitats without disturbing or restricting their interactions with host plants. We tagged the root feeding larvae of Melolontha melolontha with a copper ring and repeatedly located their position in relation to their preferred host plant, Taraxacum officinale, using a commercial metal detector...
March 16, 2017: Journal of Chemical Ecology
https://www.readbyqxmd.com/read/28303424/inherited-disorders-of-transition-metal-metabolism-an-update
#20
REVIEW
Peter T Clayton
Elements with a biological role include six trace transition metals: manganese, iron, cobalt, copper, zinc and molybdenum. Transition metals participate in group transfer reactions such as glycosylation and phosphorylation and those that can transfer an electron by alternating between two redox states such as iron (3+/2+) and copper (2+/1+) are also very important in biological redox reactions including the reduction of molecular oxygen and the transport of oxygen. However, these trace metals are also potentially toxic, generating reactive oxygen species through Fenton chemistry...
March 16, 2017: Journal of Inherited Metabolic Disease
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