Aceruloplasminemia: molecular characterization of this disorder of iron metabolism.

Ceruloplasmin is an abundant alpha 2-serum glycoprotein that contains 95% of the copper found in the plasma of vertebrate species. We report here on the identification of a genetic defect in the ceruloplasmin gene in a patient previously noted to have a total absence of circulating serum ceruloplasmin in association with late-onset retinal and basal ganglia degeneration. In this patient T2 (transverse relaxation time)-weighted magnetic resonance imaging of the brain revealed basal ganglia densities consistent with iron deposition, and liver biopsy confirmed the presence of excess iron. Although Southern blot analysis of the patient's DNA was normal, PCR amplification of 18 of the 19 exons composing the human ceruloplasmin gene revealed a distinct size difference in exon 7. DNA sequence analysis of this exon revealed a 5-bp insertion at amino acid 410, resulting in a frame-shift mutation and a truncated open reading frame. The validity of this mutation was confirmed by analysis of DNA from the patient's daughter, which revealed heterozygosity for this same 5-bp insertion. The presence of this mutation in conjunction with the clinical and pathologic findings demonstrates an essential role for ceruloplasmin in human biology and identifies aceruloplasminemia as an autosomal recessive disorder of iron metabolism. These findings support previous studies that identified ceruloplasmin as a ferroxidase and are remarkably consistent with recent studies on the essential role of a homologous copper oxidase in iron metabolism in yeast. The clinical and laboratory findings suggest that additional patients with movement disorders and nonclassical Wilson disease should be examined for ceruloplasmin gene mutations.

Comportamento em desgaste por erosão cavitação, erosão - corrosão e em ensaios de microesclerometria linear instrumentada de um aço inoxidável martensítico AISI 410 nitretado a plasma em baixa temperatura, utilizando a tecnologia de tela ativa.

Amostras de um aço inoxidável martensítico AISI 410 temperado e revenido foram nitretadas a plasma em baixa temperatura usando o tratamento de nitretação plasma DC e a nitretação a plasma com tela ativa. Ambos os tratamentos foram realizados a 400 °C, utilizando mistura gasosa de 75 % de nitrogênio e 25 % de hidrogênio durante 20 horas e 400 Pa de pressão. As amostras de aço AISI 410 temperado e revenido foram caracterizadas antes e depois dos tratamentos termoquímicos, usando as técnicas de microscopia óptica, microscopia eletrônica de varredura, medidas de microdureza, difração de raios X e medidas de teor de nitrogênio em função da distância à superfície por espectrometria WDSX de raios X. A resistência à erosão por cavitação do aço AISI 410 nitretado DC e com tela ativa foi avaliada segundo a norma ASTM G32 (1998). Os ensaios de erosão, de erosão - corrosão e de esclerometria linear instrumentada segundo norma ASTM C1624 (2005) somente foram realizados no aço AISI 410 nitretado com tela ativa. Ensaios de nanoindentação instrumentada forma utilizados para medir a dureza (H) e o módulo de elasticidade reduzido (E*) e calcular as relações H/E* e H3/E*2 e a recuperação elástica (We), utilizando o método proposto por Oliver e Pharr. Ambos os tratamentos produziram camadas nitretadas de espessura homogênea constituídas por martensita expandida supersaturada em nitrogênio e nitretos de ferro com durezas superiores a 1200 HV, porém, a nitretação DC produziu maior quantidade de nitretos de ferro do que o tratamento de tela ativa. Os resultados de erosão por cavitação do aço nitretado DC mostraram que a precipitação de nitretos de ferro é prejudicial para a resistência à cavitação já que reduziu drasticamente o período de incubação e aumentou a taxa de perda de massa nos estágios iniciais do ensaio; entretanto, depois da remoção desses nitretos de ferro, a camada nitretada formada somente por martensita expandida resistiu bem ao dano por cavitação. Já no caso do aço nitretado com tela ativa, a resistência à erosão por cavitação aumentou 27 vezes quando comparada com o aço AISI 410 sem nitretar, fato atribuído à pequena fração volumétrica e ao menor tamanho dos nitretos de ferro presente na camada nitretada, às maiores relações H/E* e H3/E*2 e à alta recuperação elástica da martensita expandida. A remoção de massa ocorreu, principalmente, pela formação de crateras e de destacamento de material da superfície dos grãos por fratura frágil sem evidente deformação plástica. As perdas de massa acumulada mostradas pelo aço nitretado foram menores do que aquelas do aço AISI 410 nos ensaios de erosão e de erosão corrosão. O aço nitretado apresentou uma diminuição nas taxas de desgaste em ambos os ensaios de aproximadamente 50 % quando comparadas com o aço AISI 410. O mecanismo de remoção de material foi predominantemente dúctil, mesmo com o grande aumento na dureza. Os resultados de esclerometria linear instrumentada mostraram que a formação de martensita expandida possibilitou uma diminuição considerável do coeficiente de atrito em relação ao observado no caso do aço AISI 410 sem nitretar. O valor de carga crítica de falha foi de 14 N. O mecanismo de falha operante no aço nitretado foi trincamento por tensão.

Iron isotope and chemical compositions of sediments and basalts drilled seaward of the Mariana Trench

The purpose of this work is to study the mobility and budget of Fe isotopes in the oceanic crust and in particular during low-temperature interaction of seawater with oceanic basalt. We carried out this investigation using samples from Ocean Drilling Program (ODP) Site 801C drilled during Leg 129 and Leg 185 in Jurassic Pacific oceanic crust seaward of the Mariana Trench. The site comprises approximately 450 m of sediment overlying a section of 500 m of basalt, which includes intercalated pelagic and chemical sediments in the upper basaltic units and two low-temperature (10-30°C) ocherous Si-Fe hydrothermal deposits. Fe was chemically separated from 70 selected samples, and 57Fe/54Fe ratios were measured by MC-ICP-MS Isoprobe. The isotopic ratios were measured relative to an internal standard solution and are reported relative to the international Fe-standard IRMM-14. Based on duplicate measurements of natural samples, an external precision of 0.2? (2 sigma) has been obtained. The results indicate that the deep-sea sediment section has a restricted range of d57Fe, which is close to the igneous rock value. In contrast, large variations are observed in the basaltic section with positive d57Fe values (up to 2.05?) for highly altered basalts and negative values (down to ?2.49?) for the associated alteration products and hydrothermal deposits. Secondary Fe-minerals, such as Fe-oxyhydroxides or Fe-bearing clays (celadonite and saponite), have highly variable d57Fe values that have been interpreted as resulting from the partial oxidation of Fe(2+) leached during basalt alteration and precipitated as Fe(3+)-rich minerals. In contrast, altered basalts at Site 801C, which are depleted in Fe (up to 80%), display an increase in d57Fe values relative to fresh values, which suggest a preferential leaching of light iron during alteration. The apparent fractionation factor between dissolved Fe(2+) and Fe remaining in the mineral is from 0.5? to 1.3? and may be consistent with a kinetic isotope fractionation where light Fe is stripped from the minerals. Alternatively, the formation of secondary clays minerals, such as celadonite during basalt alteration may incorporate preferentially the heavy Fe isotopes, resulting in the loss of light Fe isotopes in the fluids. Because microbial processes within the oceanic crust are of potential importance in controlling rates of chemical reactions, Fe redox state and Fe-isotope fractionation, we evaluated the possible effect of this deep biosphere on Fe-isotope signatures. The Fe-isotope systematics presented in this study suggest that, even though iron behavior during seafloor weathering may be mediated by microbes, such as iron-oxidizers, d57Fe variations of more than 4? may also be explained by abiotic processes. Further laboratory experiments are now required to distinguish between various processes of Fe-isotope fractionation during seafloor weathering.

Parasitic infections and storage iron deficiency in children in improverished regions of Mauritius

Using a combination of techniques including relative deprivation index developed by the Government, school performance, household survey and drawing of lots, a sample frame constituting of poverty areas of Mauritius was constructed and four test areas identified. Relevant haematological, parasitological and biochemical parameters of all school-going children living in the four test areas were determined so as to study the possibility of correlation between parasitic infections, plasma ferritin, haemoglobin concentration, white blood cells count, packed cells volume and blood group. It was found that there is a negative correlation between the number of parasites and haemoglobin concentration, packed cells volume of blood and degree of infestation, number of parasites and ferritin and number of parasites and age of subject. It has also been found that, children with blood group 'A' and blood group '0' tend to harbour the most parasites. As regards to storage iron depletion, this is significant only with hookworm infestation. Additionally it has been noted that hookworm infestation is directly related to age contrary to other parasitic infestations.

Elemental analysis of biological matrices by laser ablation high resolution inductively coupled plasma mass spectrometry (LA-HR-ICP-MS) and high resolution inductively coupled plasma mass spectrometry (HR -ICP -MS)

The need for elemental analysis of biological matrices such as bone, teeth, and plant matter for sourcing purposes has emerged within the forensic and geochemical laboratories. Trace elemental analyses for the comparison of materials such as glass by inductively coupled plasma mass spectrometry (ICP-MS) and laser ablation ICP-MS has been shown to offer a high degree of discrimination between different manufacturing sources. Unit resolution ICP-MS instruments may suffer from some polyatomic interferences including 40Ar16O+, 40Ar 16O1H+, and 40Ca 16O+ that affect iron measurement at trace levels. Iron is an important element in the analysis of glass and also of interest for the analysis of several biological matrices. A comparison of the analytical performance of two different ICP-MS systems for iron analysis in glass for determining the method detection limits (MDLs), accuracy, and precision of the measurement is presented. Acid digestion and laser ablation methods are also compared. Iron polyatomic interferences were reduced or resolved by using dynamic reaction cell and high resolution ICP-MS. MDLs as low as 0.03 μg g-1 and 0.14 μg g-1 for laser ablation and solution based analyses respectively were achieved. The use of helium as a carrier gas demonstrated improvement in the detection limits of both iron isotopes (56Fe and 57Fe) in medium resolution for the HR-ICP-MS and with a dynamic reaction cell (DRC) coupled to a quadrupole ICP-MS system. ^ The development and application of robust analytical methods for the quantification of trace elements in biological matrices has lead to a better understanding of the potential utility of these measurements in forensic chemical analyses. Standard reference materials (SRMs) were used in the development of an analytical method using HR-ICP-MS and LA-HR-ICP-MS that was subsequently applied on the analysis of real samples. Bone, teeth and ashed marijuana samples were analyzed with the developed method. ^ Elemental analysis of bone samples from 12 different individuals provided discrimination between individuals, when femur and humerus bones were considered separately. Discrimination of 14 teeth samples based on elemental composition was achieved with the exception of one case where samples from the same individual were not associated with each other. The discrimination of 49 different ashed plant (cannabis) samples was achieved using the developed method. ^

(Table 2, Page 106-107) Composition of the HCl-soluble fraction in Barents Sea iron-manganese deposits

Ferromanganese concretions from ten stations in the Barents Sea have been analysed for 24 elements. The deposits occur as discoidal and flat concretions and as coatings, in the latter case on lithified or detrital material or as extensive pavements on the Svalbard shelf. The concretions are compositionally similar to Baltic concretions but differ considerably from deep-ocean nodules, particularly in Cu, Ni and Co contents. Statistical analyses reveal distinct correlations between Mn, Na, Ba, Ni and Cu; the Mn-rich coatings showed enrichment of Mo, Zn and possibly Co in a Mn-phase. The iron phase holds high concretions of P and As. Two iron-rich concretions with high contents of P, Ca, Sr, Y, Yb and La were found east and northeast of Spitsbergen Banken, probably indicating upwelling of nutrient-rich, cold polar water along the Svalbard shelf.

An experimental geochemical characterization analysis of archaeological iron from the contact period of Newfoundland and Labrador

This research uses scanning electron microscopy-energy dispersive X-ray spectrometry (SEM-EDX) and inductively coupled plasma-mass spectrometry (ICP-MS) on cross-sections of iron artifacts sectioned from along shafts to determine the elemental constituents of a collection of Inuit and European artifacts from along the coast of Labrador. Hand-wrought iron nails from early historic period (16th – 18th centuries CE) Inuit sites in Labrador were originally manufactured by and acquired from early whalers and fishers of various European nationalities. The purpose of this research was to assess if the elements in different samples are sufficiently homogeneous to be viable for a provenience analysis to discern which Inuit nails were originally derived from which European groups; the Basque, English or French. The consistent relationships between the geochemical signatures of iron nails found in Inuit sites and historic nails derived from specific European groups could provide insights into the prevalence, activity and the nature of indigenous interactions of different European nationalities in the region over time. The results show that the methods applied to evaluate the geochemistry of the nails was not sufficient to detect meaningful patterns because the nails did not demonstrate the necessary degree of chemical uniformity among different samples in the same artifacts.

Elemental Analysis of Biological Matrices by Laser Ablation High Resolution Inductively Coupled Plasma Mass Spectrometry (LA-HR-ICP-MS) and High Resolution Inductively Coupled Plasma Mass Spectrometry (HR-ICP-MS)

The need for elemental analysis of biological matrices such as bone, teeth, and plant matter for sourcing purposes has emerged within the forensic and geochemical laboratories. Trace elemental analyses for the comparison of aterials such as glass by inductively coupled plasma mass spectrometry (ICP-MS) and laser ablation ICP-MS has been shown to offer a high degree of discrimination between different manufacturing sources. Unit resolution ICP-MS instruments may suffer from some polyatomic interferences including 40Ar16O+, 40Ar16O1H+, and 40Ca16O+ that affect iron measurement at trace levels. Iron is an important element in the analysis of glass and also of interest for the analysis of several biological matrices. A comparison of the nalytical performance of two different ICP-MS systems for iron analysis in glass for determining the method detection limits (MDLs), accuracy, and precision of the measurement is presented. Acid digestion and laser ablation methods are also compared. Iron polyatomic interferences were reduced or resolved by using dynamic reaction cell and high resolution ICP-MS. MDLs as low as 0.03 ìg g-1 and 0.14 ìg g-1 for laser ablation and solution based analyses respectively were achieved. The use of helium as a carrier gas demonstrated improvement in the detection limits of both iron isotopes (56Fe and 57Fe) in medium resolution for the HR-ICP-MS and with a dynamic reaction cell (DRC) coupled to a quadrupole ICP-MS system. The development and application of robust analytical methods for the quantification of trace elements in biological matrices has lead to a better understanding of the potential utility of these measurements in forensic chemical analyses. Standard reference materials (SRMs) were used in the development of an analytical method using HR-ICP-MS and LA-HR-ICP-MS that was subsequently applied on the analysis of real samples. Bone, teeth and ashed marijuana samples were analyzed with the developed method. Elemental analysis of bone samples from 12 different individuals provided discrimination between individuals, when femur and humerus bones were considered separately. Discrimination of 14 teeth samples based on elemental composition was achieved with the exception of one case where samples from the same individual were not associated with each other. The discrimination of 49 different ashed plant (cannabis)samples was achieved using the developed method.

Iron assimilation by the clam Laternula elliptica from Potter Cove, King Georg Island, Antarctica

Iron stable isotope signatures (d56Fe) in hemolymph (bivalve blood) of the Antarctic bivalve Laternula elliptica were analyzed by Multiple Collector-Inductively Coupled Plasma-Mass Spectrometry (MC-ICP-MS) to test whether the isotopic fingerprint can be tracked back to the predominant sources of the assimilated Fe. An earlier investigation of Fe concentrations in L. elliptica hemolymph suggested that an assimilation of reactive and bioavailable Fe (oxyhydr)oxide particles (i.e. ferrihydrite), precipitated from pore water Fe around the benthic boundary, is responsible for the high Fe concentration in L. elliptica (Poigner et al., 2013, doi:10.1016/j.ecss.2013.10.027). At two stations in Potter Cove (King George Island, Antarctica) bivalve hemolymph showed mean d56Fe values of -1.19 ± 0.34 per mil and -1.04 ± 0.39 per mil, respectively, which is between 0.5 per mil and 0.85 per mil lighter than the pool of easily reducible Fe (oxyhydr)oxides of the surface sediments (-0.3 per mil to -0.6 per mil). This is in agreement with the enrichment of lighter Fe isotopes at higher trophic levels, resulting from the preferential assimilation of light isotopes from nutrition. Nevertheless, d56Fe hemolymph values from both stations showed a high variability, ranging between -0.21 per mil (value close to unaltered/primary Fe(oxyhydr)oxide minerals) and -1.91 per mil (typical for pore water Fe or diagenetic Fe precipitates), which we interpret as a "mixed" d56Fe signature caused by Fe assimilation from different sources with varying Fe contents and d56Fe values. Furthermore, mass dependent Fe fractionation related to physiological processes within the bivalve cannot be ruled out. This is the first study addressing the potential of Fe isotopes for tracing back food sources of bivalves.

Departure of high temperature iron lines from the equilibrium state in flaring solar plasmas

The aim of this study is to clarify if the assumption of ionization equilibrium and a Maxwellian electron energy distribution is valid in flaring solar plasmas. We analyze the 2014 December 20 X1.8 flare, in which the \ion{Fe}{xxi} 187~\AA, \ion{Fe}{xxii} 253~\AA, \ion{Fe}{xxiii} 263~\AA\ and \ion{Fe}{xxiv} 255~\AA\ emission lines were simultaneously observed by the EUV Imaging Spectrometer onboard the Hinode satellite. Intensity ratios among these high temperature Fe lines are compared and departures from isothermal conditions and ionization equilibrium examined. Temperatures derived from intensity ratios involving these four lines show significant discrepancies at the flare footpoints in the impulsive phase, and at the looptop in the gradual phase. Among these, the temperature derived from the \ion{Fe}{xxii}/\ion{Fe}{xxiv} intensity ratio is the lowest, which cannot be explained if we assume a Maxwellian electron distribution and ionization equilibrium, even in the case of a multi-thermal structure. This result suggests that the assumption of ionization equilibrium and/or a Maxwellian electron energy distribution can be violated in evaporating solar plasma around 10MK.

Iron mineral structure, reactivity, and isotopic composition in a South Pacific Gyre ferromanganese nodule over 4 Ma

Deep-sea ferromanganese nodules accumulate trace elements from seawater and underlying sediment porewaters during the growth of concentric mineral layers over millions of years. These trace elements have the potential to record past ocean geochemical conditions. The goal of this study was to determine whether Fe mineral alteration occurs and how the speciation of trace elements responds to alteration over ∼3.7 Ma of marine ferromanganese nodule (MFN) formation, a timeline constrained by estimates from 9Be/10Be concentrations in the nodule material. We determined Fe-bearing phases and Fe isotope composition in a South Pacific Gyre (SPG) nodule. Specifically, the distribution patterns and speciation of trace element uptake by these Fe phases were investigated. The time interval covered by the growth of our sample of the nodule was derived from 9Be/10Be accelerator mass spectrometry (AMS). The composition and distribution of major and trace elements were mapped at various spatial scales, using micro-X-ray fluorescence (μXRF), electron microprobe analysis (EMPA), and inductively coupled plasma mass spectrometry (ICP-MS). Fe phases were characterized by micro-extended X-ray absorption fine structure (μEXAFS) spectroscopy and micro-X-ray diffraction (μXRD). Speciation of Ti and V, associated with Fe, was measured using micro-X-ray absorption near edge structure (μXANES) spectroscopy. Iron isotope composition (δ56/54Fe) in subsamples of 1-3 mm increments along the radius of the nodule was determined with multiple-collector ICP-MS (MC-ICP-MS). The SPG nodule formed through primarily hydrogeneous inputs at a rate of 4.0 ± 0.4 mm/Ma. The nodule exhibited a high diversity of Fe mineral phases: feroxyhite (δ-FeOOH), goethite (α-FeOOH), lepidocrocite (γ-FeOOH), and poorly ordered ferrihydrite-like phases. These findings provide evidence that Fe oxyhydroxides within the nodule undergo alteration to more stable phases over millions of years. Trace Ti and V were spatially correlated with Fe and found to be adsorbed to Fe-bearing minerals. Ti/Fe and V/Fe ratios, and Ti and V speciation, did not vary along the nodule radius. The δ56/54Fe values, when averaged over sample increments representing 0.25 to 0.75 Ma, were homogeneous within uncertainty along the nodule radius, at -0.12 ± 0.07 ‰ (2sd, n=10). Our results indicate that the Fe isotope composition of the nodule remained constant during nodule growth and that mineral alteration did not affect the primary Fe isotope composition of the nodule. Furthermore, the average δ56/54Fe value of -0.12 ‰ we find is consistent with Fe sourced from continental eolian particles (dust). Despite mineral alteration, the trace element partitioning of Ti and V, and Fe isotope composition, do not appear to change within the sensitivity of our measurements. These findings suggest that Fe oxyhydroxides within hydrogenetic ferromanganese nodules are out of geochemical contact with seawater once they are covered by subsequent concentric mineral layers. Even though Fe-bearing minerals are altered, trace element ratios, speciation and Fe isotope composition are preserved within the nodule.

Iron Transformation Pathways and Redox Micro-Environments in Seafloor Sulfide-Mineral Deposits: Spatially Resolved Fe XAS and delta Fe-57/54 Observations

Hydrothermal sulfide chimneys located along the global system of oceanic spreading centers are habitats for microbial life during active venting. Hydrothermally extinct, or inactive, sulfide deposits also host microbial communities at globally distributed sites. The main goal of this study is to describe Fe transformation pathways, through precipitation and oxidation-reduction (redox) reactions, and examine transformation products for signatures of biological activity using Fe mineralogy and stable isotope approaches. The study includes active and inactive sulfides from the East Pacific Rise 9 degrees 50'N vent field. First, the mineralogy of Fe(III)-bearing precipitates is investigated using microprobe X-ray absorption spectroscopy (RXAS) and X-ray diffraction (mu XRD). Second, laser-ablation (LA) and micro-drilling (MD) are used to obtain spatially-resolved Fe stable isotope analysis by multicollector-inductively coupled plasma-mass spectrometry (MC-ICP-MS). Eight Fe -bearing minerals representing three mineralogical classes are present in the samples: oxyhydroxides, secondary phyllosilicates, and sulfides. For Fe oxyhydroxides within chimney walls and layers of Si-rich material, enrichments in both heavy and light Fe isotopes relative to pyrite are observed, yielding a range of delta Fe-57 values up to 6 parts per thousand. Overall, several pathways for Fe transformation are observed. Pathway 1 is characterized by precipitation of primary sulfide minerals from Fe(II)aq-rich fluids in zones of mixing between vent fluids and seawater. Pathway 2 is also consistent with zones of mixing but involves precipitation of sulfide minerals from Fe(II)aq generated by Fe(III) reduction. Pathway 3 is direct oxidation of Fe(II) aq from hydrothermal fluids to form Fe(III) precipitates. Finally, Pathway 4 involves oxidative alteration of pre-existing sulfide minerals to form Fe(III). The Fe mineralogy and isotope data do not support or refute a unique biological role in sulfide alteration. The findings reveal a dynamic range of Fe transformation pathways consistent with a continuum of micro-environments having variable redox conditions. These micro-environments likely support redox cycling of Fe and S and are consistent with culture-dependent and -independent assessments of microbial physiology and genetic diversity of hydrothermal sulfide deposits.

Determinação de metais em óleos lubrificantes utilizando a técnica de espectrometria de emissão óptica com plasma induzido por laser

IPEN/D

Influence of magnesium on biochemical parameters of iron and oxidative stress in patients with type 2 diabetes

Introduction: Studies have shown that oxidative stress, found in patients with type 2 diabetes, may be due to changes in the metabolism of minerals, such as magnesium and iron. Data related to compartmentalization of these minerals in diabetes are scarce and controversial. Objective: This study assessed the influence of magnesium on biochemical parameters of iron and oxidative stress in patients with type 2 diabetes. Methods: A case-control study in male and female subjects aged 27-59 years, divided into two groups: type 2 diabetes (n=40) and control (n=48). Intake of magnesium and iron was assessed by three-day food record. Plasma, erythrocyte and urinary levels of magnesium, serum iron, ferritin, total iron binding capacity, fasting glucose, glycated hemoglobin, insulin, creatinine clearance and plasma thiobarbituric acid reactive substances (TBARS) were analyzed. Results and Discussion: Magnesium intake and plasma magnesium were lower in diabetic subjects. There was low urinary magnesium excretion, with no difference between groups. Although normal, the diabetic group had lower serum iron and ferritin concentrations compared to control subjects. Plasma TBARS in diabetic patients was higher than control while creatinine clearance was lower. An inverse correlation between erythrocyte magnesium and serum iron and ferritin was observed in the diabetes group. Conclusions: Diabetes induced hypomagnesemia and this, associated with chronic hyperglycemia, may have enhanced oxidative stress. Erythrocyte magnesium may have contributed to prevent iron overload and worsening of oxidative stress and hyperglycemic status.