119 resultados para Carbamyl Phosphate Synthase Deficiency


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The complete amino-acid sequence of sheep liver cytosolic serine hydroxymethyltransferase was determined from an analysis of tryptic, chymotryptic, CNBr and hydroxylamine peptides. Each subunit of sheep liver serine hydroxymethyltransferase consisted of 483 amino-acid residues. A comparison of this sequence with 8 other serine hydroxymethyltransferases revealed that a possible gene duplication event could have occurred after the divergence of animals and fungi. This analysis also showed independent duplication of SHMT genes in Neurospora crassa. At the secondary structural level, all the serine hydroxymethyltransferases belong to the alpha/beta category of proteins. The predicted secondary structure of sheep liver serine hydroxymethyltransferase was similar to that of the observed structure of tryptophan synthase, another pyridoxal 5'-phosphate containing enzyme, suggesting that sheep liver serine hydroxymethyltransferase might have a similar pyridoxal 5'-phosphate binding domain. In addition, a conserved glycine rich region, G L Q G G P, was identified in all the serine hydroxymethyltransferases and could be important in pyridoxal 5'-phosphate binding. A comparison of the cytosolic serine hydroxymethyltransferases from rabbit and sheep liver with other proteins sequenced from both these sources showed that serine hydroxymethyltransferase was a highly conserved protein. It was slightly less conserved than cytochrome c but better conserved than myoglobin, both of which are well known evolutionary markers. C67 and C203 were specifically protected by pyridoxal 5'-phosphate against modification with [C-14]iodoacetic acid, while C247 and C261 were buried in the native serine hydroxymethyltransferase. However, the cysteines are not conserved among the various serine hydroxymethyltransferases. The exact role of the cysteines in the reaction catalyzed by serine hydroxymethyltransferase remains to be elucidated.

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The direct intratesticular injection of [P-32]phosphate resulted in 4-9 times more labelling of rat testis proteins compared to the conventional method of in vitro incubation. Moreover this is a simple technique requiring minimum (7-10 times less) radioactive phosphate and is less hazardous.

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In this paper, the effect of phosphate anion adsorption on the permeability values of homoionized kaolinite and montmorillonite clays is presented. The homoionized sodium, calcium and hydrogen clays are prepared by repeatedly washing the clays with 2N solutions of corresponding chlorides. Phosphate adsorption was induced by treating homoionized clays with phosphoric acids for different periods varying upto 1000 hrs. The coefficient of permeability of the clays was determined from one dimensional consolidation test results. The decrease in the permeability of kaolinite clays on phosphate adsorption has been explained on the fabric changes. For montmorillonite, both cation exchange and phosphate adsorption causes significant changes which are explained based on variation in the thickness of diffuse double layer.

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By employing EXAFS and magnetic measurements, it is shown that nanoparticles of nickel along with those of NiO are incorporated between the layers of a-zirconium phosphate (ZrP) by the thermal decomposition of nickel acetate intercalated in ZrP. The nickel nanoparticles are superparamagnetic. Hydrogen reduction produces small ferromagnetic nickel particles, most of which appear to be outside the interlayer space of ZrP.

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Sheep liver serine hydroxymethyltransferase (EC 2.1.2.1) is a homotetramer of M(r) 213,000 requiring pyridoxal-5'-phosphate (PLP) as cofactor, Removal of PLP from the holoenzyme converted the enzyme to the apo form which, in addition to being inactive, was devoid of the characteristic absorption spectrum. Upon the addition of PLP to the apoenzyme, complete activity was restored and the visible absorption spectrum with a maximum at 425 nm was regained. The interaction of PLP with the apoenzyme revealed two phases of reaction with pseudo-first-order rate constants of 20 +/- 5 s(-1) and 12.2 +/- 2.0 x 10(-3) s(-1), respectively. However, addition of PLP to the apoenzyme did not cause gross conformational changes as evidenced by circular dichroic and fluorescence spectroscopy. Although conformationally apoenzyme and holoenzyme were indistinguishable, they had distinct apparent melting temperatures of 51 +/- 2 and 58 +/- 2 degrees C, respectively, and the reconstituted holoenzyme was thermally as stable as the native holoenzyme. These results suggested that there was no apparent difference in the secondary structure of holoenzyme, apoenzyme, and reconstituted holoenzyme, However, sedimentation analysis of the apoenzyme revealed the presence of two peaks of S-20,S-w values of 8.7 +/- 0.5 and 5.7 +/- 0.3 S, respectively. A similar pattern was observed when the apoenzyme was chromatographed on a calibrated Sephadex G-150 column. The first peak corresponded to the tetrameric form (M(r) 200,000 +/- 15,000) while the second peak had a M(r) of 130,000 +/- 10,000. Reconstitution experiments revealed that only the tetrameric form of the apoenzyme could be converted into an active holoenzyme while the dimeric form could not be reconstituted into an active enzyme. These results demonstrate that PLP plays an important role in maintaining the structural integrity of the enzyme by preventing the dissociation of the enzyme into subunits, in addition to its function in catalysis. (C) 1996 Academic Press, Inc.

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Partial substitution of Cu in the chain by the phosphate ion stabilizes LnSr(2)Cu(3)O(7) (Ln = Gd, Dy or Ho) in the 123 structure. The LnSr(2)Cu(2.8)(PO4)(0.2)O-y derivatives exhibit incommensurately modulated structures. The holmium oxy-phosphate derivative has been rendered superconducting by the partial substitution of Ho by Ca.

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Glasses of the alkali tin phosphate system have been investigated. The infrared absorption and fluorescence spectra of the glasses have been examined. It is found that tin is present in both + 2 and + 4 oxidation states. Also tin ions occupy four- or six-coordinated sites in the glass.

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Five new open-framework compounds of gallium have been synthesized by hydrothermal methods and their structures determined by single crystal X-ray diffraction studies. The compounds, C8N4H26]Ga6F4(PO4)(6)], I, C5N3H11]Ga3F2(PO4)(3)]center dot H2O, II, C6N3H19]Ga-4(C2O4)(PO4)(4)(H2PO4)]center dot 2H(2)O, III, Ga2F3(HPO4)(PO4)]center dot 2H(3)O, IV, and C3N2H5](2)Ga-4(H2O)(3)(HPO3)(7)], V, possess three-dimensional structures. All the compounds are formed by the connectivity between the Ga polyhedra and phosphite/phosphate units. The observation of SBU-6 (I and II) and spiro-5 (IV) secondary building units (SBUs) are noteworthy. The flexibility of the formation of gallium phosphate frameworks has been established by the isolation of two related structures (I and II) from the same SBU units but different organic amines. Some of the present structures have close resemblance to the gallium phosphate phases known earlier. The compounds have been characterized by CHN analysis, powder XRD, IR, and TGA. (C) 2011 Elsevier B. V. All rights reserved.

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Reported distress to an industrial structure from phosphate/sulfate contamination of kaolinitic foundation soil at an industrial location in Southern India prompted this laboratory study. The study examines the short-term effect of sodium sulfate/phosphate contamination on the swell/compression characteristics of a commercial kaolinite. Experimental results showed that the unsaturated contaminated kaolinite specimens exhibited slightly higher swell potentials and lower compressions than the unsaturated uncontaminated kaolinite specimens. It is suggested that the larger double layer promoted by the increased exchangeable sodium ion concentration is responsible for the slightly higher swell potentials and lower compressions of the unsaturated contaminated kaolinite specimens.

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A new series of multielement flame-retardant plasticizers containing polyethylene stibinite phosphate esters have been prepared by bulk polymerization from ethylene glycol with various antimony (III) aryloxydichlorides and arylphosphorodichloridates possessing various combinations of substituent [Cl,Br,NO2]. All the polymers are pink-coloured viscous fluids. They were characterized by inherent viscosity, density, IR, H-1, C-13 and P-31 NMR spectroscopy. The thermal behaviour of the polymers was compared by thermogravimetric analysis and correlated with their structures. The flammability studies were carried out by the limiting oxygen index test. The polymers containing P, Sb, N and Pr elements in their backbone show superior thermal-and flame-retardant characteristics than the other polymers. A comparative study was carried out with one of the synthesized polymers as a polymeric flame-retardant additive to plasticized PVC. The results showed improved LOI and mechanical properties to that of the conventional flame-retardant additive composition. (C) 1997 Elsevier Science Ltd.

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Dimeric or gemini surfactants consist of two hydrophobic chains and two hydrophilic head groups covalently connected by a hydrophobic or hydrophilic spacer. This paper reports the small-angle neutron scattering (SANS) measurements from aqueous micellar solutions of two different recently developed types of dimeric surfactants: (i) bis-anionic C16H33PO4--(CH2)(m)-PO4-C16H33,2Na(+) dimeric surfactants composed of phosphate head groups and a hydrophobic polymethylene spacer, referred to as 16-m-16,2Na(+), for spacer lengths m = 2, 4, 6, and 10, (ii) bis-cationic C16H33N+(CH3)(2)-CH2-(CH2-O-CH2)(p)-CH2-N+ (CH3)(2)C16H33,2Br(-) dimeric surfactants composed of dimethylammonium head groups and a wettable polyethylene oxide spacer, referred to as 16-CH2-p-CH2-16,2Br(-), for spacer lengths p = 1 - 3. The micellar structures of these surfactants are compared with the earlier studied bis-cationic C16H33N+ (CH3)(2)-(CH2)(m)-N+ (CH3)(2)C16H33,2Br(-) dimeric surfactants composed of dimethylammonium head groups and a hydrophobic polymethylene spacer, referred to as 16-m-16,2Br(-). It is found that 16-m-16,2Na(+), similar to 16-m-16,2Br(-), form various micellar structures depending on the spacer length. Micelles an disklike for rn = 2, rodlike for m = 4, and prolate ellipsoidal fur m = 6 and 10. The micelles of 16-CH2-p-CH2-16,2Br(-) are prolate ellipsoidal for all the values of p = 1 - 3. It is also found that micelles of 16-m-16,2Na(+) and 16-CH2-p-CH2-16,2Br(-) are large in comparison to those of 16-in-16,2Br(-) for similar spacer lengths. This is connected with the fact that both in 16-m-16,2Na(+) and 16-CH2-p-CH2-16,2Br(-), the head group or the spacer is more hydrated as compared to that in the 16-m-16,2Br(-). An increase in the hydration of the spacer or the head group increases the screening of the Coulomb repulsion between the charged head groups. This effect has been found to be more pronounced in the dimeric surfactants having wettable spacers. [S1063-651X(99)00303-7].

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Potassium titanyl phosphate (KTP) and its isomorphs have received enormous attention in the last 2 decades. In particular, KTP assumes importance due to its large nonlinear optic and electrooptic coefficients together with the broad thermal and angular acceptance for second harmonic generation. This article provides an overview of the material aspects, structural, physical, and chemical properties and device feasibility of the KTP family of crystals. Some of the current areas of research and development along with their significance in understanding the physical properties as well as device applications are addressed. Optical waveguide fabrication processes and characteristics with their relevance to the present-day technology are highlighted. Studies performed so far have enabled us to understand the fundamental aspects of these materials and what needs to be pursued vigorously is the exploitation of their device applications to the maximum extent.

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Marked ball grinding rests were carried out in the laboratory with a low grade phosphate ore under different experimental conditions. Two types of balls were used, namely high carbon low alloy (HCLA) cast steel and high chrome cast iron. Results of marked ball grinding tests indicated that ball wear increased with time and showed a sharp increase for wet grinding over dry grinding. Ball wear under wet grinding conditions was also influenced by the gaseous atmosphere in the mill. The grinding ball materials could be arranged in the following order with respect to their overall wear resistance: High chrome cast iron > HCLA cast steel balls Methods to minimize ball wear through control of mill atmosphere and addition of flotation reagents are discussed. Effect of grinding media and additions of flotation reagents during grinding on phosphate ore flotation are also discussed. (C) 1999 Elsevier Science Ltd. All rights reserved.