22 research outputs found

    A concurrent error detection based fault-tolerant 32 nm XOR-XNOR circuit implementation

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    As modern processors and semiconductor circuits move into 32 nm technologies and below, designers face the major problem of process variations. This problem makes designing VLSI circuits harder and harder, affects the circuit performance and introduces faults that can cause critical failures. Therefore, fault-tolerant design is required to obtain the necessary level of reliability and availability especially for safety-critical systems. Since XOR-XNOR circuits are basic building blocks in various digital and mixed systems, especially in arithmetic circuits, these gates should be designed such that they indicate any malfunction during normal operation. In fact, this property of verifying the results delivered by a circuit during its normal operation is called Concurrent Error Detection (CED). In this paper, we propose a CED based fault- tolerant XOR-XNOR circuit implementation. The proposed design is performed using the 32 nm process technology.published_or_final_versio

    Hurler disease (mucopolysaccharidosis type IH): clinical features and consanguinity in Tunisian population

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    Mucopolysaccharidosis type I (MPS I) was a group of rare autosomal recessive disorder caused by the deficiency of the lysosomal enzyme, alpha -L -iduronidase, and the resulting accumulation of undergraded dematan sulfate and heparan sulfate. MPS I patients have a wide range of clinical presentations, that makes it difficult to predict patient phenotype which is needed for genetic counseling and also impedes the selection and evaluation of patients undergoing therapy bone marrow transplantation

    Mucopolysaccharidosis type I: molecular characteristics of two novel alpha-L-iduronidase mutations in Tunisian patients

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    <p>Abstract</p> <p>Background</p> <p>Mucopolysaccharidosis type I (MPS I) is an autosomal storage disease resulting from defective activity of the enzyme α-L-iduronidase (IDUA). This glycosidase is involved in the degradation of heparan sulfate and dermatan sulfate. MPS I has severe and milder phenotypic subtypes.</p> <p>Aim of study: This study was carried out on six newly collected MPS I patients recruited from many regions of Tunisia.</p> <p>Patients and methods: Mutational analysis of the IDUA gene in unrelated MPS I families was performed by sequencing the exons and intron-exon junctions of IDUA gene.</p> <p>Results</p> <p>Two novel IDUA mutations, p.L530fs (1587_1588 insGC) in exon 11 and p.F177S in exon 5 and two previously reported mutations p.P533R and p.Y581X were detected. The patient in family 1 who has the Hurler phenotype was homozygous for the previously described nonsense mutation p.Y581X.</p> <p>The patient in family 2 who also has the Hurler phenotype was homozygous for the novel missense mutation p.F177S. The three patients in families 3, 5 and 6 were homozygous for the p.P533R mutation. The patient in family 4 was homozygous for the novel small insertion 1587_1588 insGC. In addition, eighteen known and one unknown IDUA polymorphisms were identified.</p> <p>Conclusion</p> <p>The identification of these mutations should facilitate prenatal diagnosis and counseling for MPS I in Tunisia.</p> <p>Background</p> <p>Mucopolysaccharidosis type I (MPS I) is an autosomal recessive lysosomal storage disorder caused by the deficient activity of the enzyme of α-L-iduronidase (IDUA, EC 3.2.1.76). This glycosidase is involved in the degradation of heparan sulfate and dermatan sulfate. The clinical phenotype of MPS I ranges from the very severe in Hurler syndrome (MPS IH) to the relatively benign in Scheie syndrome (MPS IS), with an intermediate phenotype designated Hurler/Scheie (MPS IH/S) <abbrgrp><abbr bid="B1">1</abbr></abbrgrp>. Isolation of complementary and genomic DNAs encoding human α -L- iduronidase <abbrgrp><abbr bid="B2">2</abbr><abbr bid="B3">3</abbr></abbrgrp> have enable the identification of mutations underlying the enzyme defect and resulting in MPS I clinical phenotype. More than 100 mutations have been reported in patients with the MPS I subtypes (Human Gene Mutation Database; <url>http://www.hgmd.org</url>). High prevalence of the common mutations p.W402X and p.Q70X has been described; both of them in the severe clinical forms <abbrgrp><abbr bid="B4">4</abbr><abbr bid="B5">5</abbr></abbrgrp>. A high prevalence of common mutation p.P533R has also been described in MPS I patients with various phenotypes <abbrgrp><abbr bid="B5">5</abbr><abbr bid="B6">6</abbr></abbrgrp>. In addition, rare mutations including single base substitution, deletion, insertion and splicing site mutation have been identified <abbrgrp><abbr bid="B7">7</abbr></abbrgrp>, indicating a high degree of allelic heterogeneity in IDUA gene.</p> <p>Here, we described two novel IDUA mutations in MPS I Tunisian patients. These lesions were homoallelic in all the patients of the six families investigated as consanguineous marriages are still frequent in Tunisia <abbrgrp><abbr bid="B8">8</abbr></abbrgrp>.</p

    Amortissement des fluctuations de température d'un fluide circulant dans une conduite circulaire ou annulaire

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    A digital model to monitor the evolution of a thermic field inside a product flowing in a conditioning circuit has been developed. This model takes into account the circuit inertia, the circuit-product interaction. There is a good correlation between the digital model and the experimental results. / Un modèle numérique permettant le suivi de l'évolution du champ thermique au sein d'un produit traversant un circuit de conditionnement a été élaboré. Ce modèle tient compte de l'inertie du circuit, de l'interaction produit-circuit. On constate un bon accord entre le modèle numérique et les résultats expérimentaux

    Injection de vapeur dans un liquide en présence d'une accélération centrifuge

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    This is a study on a heat exchanger by direct injection of vapor in a liquid. The process consists of injecting vapor in an elbow. A centrifugal acceleration is thus created and it can reach 80 times the acceleration of gravity. / L'étude porte sur un échangeur de chaleur par injection directe de vapeur dans un liquide. Le procédé consiste à injecter la vapeur au niveau d'un coude. On crée ainsi une accélération centrifuge pouvant atteindre 80 fois l'accélération de la pesanteur

    Wedge product of currents

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    Crystal structure, quantum mechanical investigation, IR and NMR spectroscopy of two new organic perchlorates: (C6H18N3)·(ClO4)3H2O (I) and (C9H11N2)·ClO4(II)

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    The reaction of perchloric acid with 1-(2-aminoethyl)piperazine or 5,6-dimethyl-benzimidazole results in the formation of 1-(2-amonioethyl)piperazine-1,4-dium triperchlorate hydrate (C6H18N3)·(ClO4)3·H2O (I) or 5,6-dimethyl-benzylimidazolium perchlorate (C9H11N2)·ClO4(II). Both compounds were fully structurally characterized including single crystal X-ray diffraction analysis. Compound (I) crystallizes in the centrosymmetric triclinic space group P 1¯ with the lattice parameters a = 7.455 (2), b = 10.462 (2), c = 10.824 (2) Å, α = 80.832 (2), β = 88.243 (2), γ = 88.160 (2) °, Z = 2 and V = 832.77 (3) Å3. Compound (II) has been found to belong to the P21/c space group of the monoclinic system, with a = 7.590 (3), b = 9.266 (3), c = 16.503 (6) Å, β = 107.38 (2) °, V = 1107.69 (7) Å3and Z = 4. The structures of (I) and (II) consist of slightly distorted [ClO4]-tetrahedra anions and 1-(2-amonioethyl)piperazine-1,4-dium trication (I) or 5,6-dimethyl-benzylimidazolium cations (II) and additionally a lattice water in (I). The crystal structures of (I) and (II) exhibit complex three-dimensional networks of H-bonds connecting all their components. In the atomic arrangement of (I), the ClO4−anions form corrugated chains, while in (II) the atomic arrangement exhibits wide pseudo-hexagonal channels of ClO4tetrahedra including the organic entities. The lattice water serves as a link between pairs of cations and pairs of anions via several O–H⋯O and N-H⋯O interactions in compound (I). The vibrational absorption bands were identified by infrared spectroscopy. These compounds were also investigated by solid-state13C,35Cl and15N NMR spectroscopy. DFT calculations allowed the attribution of the IR and NMR bands. Intermolecular interactions were investigated by Hirshfeld surfaces. Electronic properties such as HOMO and LUMO energies were derived

    A Hirshfeld surface analysis, crystal structure and infrared characterization of a new organic cyclohexaphosphate, (C8H12N)4(Li)2(P6O18)(H2O)6

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    Single crystals of a new organic cyclohexaphosphate (C8H12N)4(Li)2(P6O18)(H2O)6 prepared by an acid/base reaction between cyclohexaphosphoric acid H6P6O18 and 2,6-dimethylaniline, have been successfully grown by slow evaporation technique at room temperature. This compound was characterized using X-ray diffraction (XRD) and FT-IR spectroscopy. The results show that the title compound was phased with a triclinic structure and P-1 space group. The crystal structure was solved by using 5203 independent reflections with a final R value of 0.031. The P6O18 ring is centrosymmetrical. Its main geometrical features are those commonly observed in others cyclohexaphosphates. The atomic arrangement of this compound can be described by an organization in a three-dimensional framework, built up from alternated organic and inorganic species. Adjacent P6O18 rings are linked via corner-sharing by LiO4 tetrahedra, generating anionic [Li2(P6O18)(H2O)]4- layers parallel to (110). Charge balance is achieved by the protonated amine which is trapped in the interlayer space and interacts with the organic framework through strong and medium hydrogen bonding. An extensive network of N[sbnd]H⋯O and O[sbnd]H⋯O hydrogen-bonding interactions link the components into a three-dimensional network and additional stabilization is provided by weak C[sbnd]H⋯O hydrogen bonds. Intermolecular interactions were investigated by Hirshfeld surfaces. The vibrational absorption bands were identified by infrared spectroscopy. The stretching modes of P6O18 ring and band assignments are described
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