Chromosome 15-linked limb-girdle muscular dystrophy: clinical phenotypes in Reunion Island and French metropolitan communities

Erb's type limb-girdle muscular dystrophy (LGMD) was identified and clinically studied in detail in a small community living in the Reunion Island (RI). It was linked to chromosome 15q and related to mutations in the muscle specific calpain 3 gene. A series of cases were afterwards clinically and genetically identified in the French metropolitan community. The phenotype was identical to the RI type in the great majority of cases, although clinical differences were noticed in a few cases. Six different mutations were identified in the RI families, whereas a series of 39 mutations were detected in the French metropolitan families, all different from those present in the RI patients. Phenotype-genotype correlations were attempted in both communities.     

Solution- and crystal-phase covalent modification of lysozyme by a purpose-designed organoruthenium complex. A MALDI-TOF MS study of its metal binding sites

Study of the reaction between the transition organometallic complex 4-ruthenocenyl 2,6-dimethylpyrylium tetrafluoroborate and the enzyme hen egg white lysozyme (HEWL) in solution and by diffusion in crystals was performed by use of a combination of spectroscopic and chromatographic methods. Conjugation involving the lysine residues of lysozyme appeared to occur readily, yielding very stable ruthenocenyl pyridinium adducts with average degrees of incorporation ranging from 0.2 to 1.8 metal complexes per protein molecule, depending on reaction conditions. Matrix-assisted laser desorption ionization time-of-flight mass spectrometry (MALDI-TOF MS) revealed that the protein conjugates were in fact mixtures of unmodified, mono-, di- and sometimes tripyridinium adducts. In combination with reversed-phased HPLC, we were able to show that six different monoruthenocenyl pyridinium adducts were formed in solution. This result was confirmed by trypsin digestion of a ruthenocenyl pyridinium conjugate and MALDI-TOF MS analysis of the peptide mixture, which showed that lysines 1, 13, 33, 96, 97 and 116 were involved in the reaction with the pyrylium complex, lysines 13, 33 and 116 being the major binding sites. In the tetragonal crystal state, no binding of the ruthenium complex was shown to occur at lysine 116, owing to steric hindrance at this particular position.     

As(V) retention and As(III) simultaneous oxidation and removal on a MnO2-loaded polystyrene resin

Based on KMnO4 oxidative capacity, a polystyrene matrix loaded with manganese dioxide was synthesized from an anionic commercial resin in chloride form. This medium, called R-MnO2, was tested for As(V) retention and for As(III) simultaneous oxidation and removal. Equilibrium was reached in 2 h and isotherms showed that R-MnO2 maximal capacities towards As(III) and As(V) are, respectively, 0.7 and 0.3 mmol/g. Various mechanisms were involved in As(III) retention: oxidation of H3AsO3(0) by MnO2(s) leading to the formation of HAsO4(2)- and Mn2+, fixation of As(V) formed on the resin beads and precipitation of Mn3(AsO4)2 with Mn2+ released. Successive arsenic desorption and retention steps were performed and showed that the quantity desorbed was low compared to the quantity removed during the first stage of the process. A second removal step, carried out under the same conditions as the first one, proved that the matrix second-removal capacity was weak. This solid sorbent, although not reusable, can be considered in field application as arsenic retention is really strong.     

PREDICTOL: a computer program to determine the thermophysiological duration limited exposures in various climatic conditions

PREDICTOL is a PC program used to determine the thermophysiological duration limited exposures (DLE) in humans, nude or clothed, submitted to various climatic conditions (hot and cold climates) at rest or during a physical exercise. DLE are determined following different standards of the International Standardization Organization (ISO), especially ISO 7933 for hot environment and ISO-TR 11079 for cold environment. The original aspect of this program is that it can be used whatever the climatic conditions. The program presents two modes: an educational interactive mode and a scenario mode. The educational interactive mode demonstrates the thermophysiological effects, expressed as DLE, of different parameter changes (temperature, humidity, wind speed, metabolic heat production by physical exercise, clothing insulation and water vapor permeability). The scenario mode determines DLE for given various linked sequences as encountered in occupational, military or even recreational activities, each sequence being characterized by its climatic conditions, physical activities performed and by physical clothing properties. DLE given by PREDICTOL are correlated to those obtained in various controlled climatic laboratory conditions (r = 0.86; P < 0.001). PREDICTOL is written in Visual Basic 6.0. A "help menu" is provided to explain the use of the program and give information concerning the equations used to calculate both the thermal balance and DLE.     

Preparation par transport en phase vapeur et caracterisation de monocristaux d' oxyde ternaire V1−xNbxOw(0 ⩽ x ⩽ 0,33)

Single crystals of V_1?xNb_xO₂ ternary oxide (0 ? x ? 0,33) are prepared by chemical transport using TeCl₄. The compositions are determined by thermogravimétric analysis under oxygen, X-ray diffraction measurements and evaluation of the insulator-metal transition temperature.     

Evaluation of IP over WDM Network Architectures

The commonly used IP-backbone network architecture of today is IP/ATM/SDH/WDM. This architecture has many redundant functionalities and is not optimized to transport IP traffic. New approaches for simplified network architectures try to eliminate redundant functionalities and to decrease the protocol overhead and thereby transport IP as efficiently as possible over WDM-based optical networks. EURESCOM project P918 Integration of IP-over-optical networks: networking and management investigated scenarios for optimized IP transport in WDM-based backbone networks. In this paper, three architectures, namely Gigabit Ethernet, Packet over Sonet/SDH and Dynamic Packet Transport are investigated and evaluated as an alternative to the IP/ATM/SDH/WDM architecture.     

升温速率对氧化铝粉末烧结行为的影响

采用不同的升温速率,在膨胀计中对脱脂后的氧化铝粉末射成形坯件进行一系列的烧结试验.结果表明,烧结致密化过程主要发生在升温阶段,快速升温有利于致密化的进行和抑止晶粒长大,但由于烧结时间较短和烧结炉最高温度的限制,产品的最终致密化程度不高.在低温时快速升温,高温时缓慢加热,可以获得较好的致密化效果和微观结构.试验和分析结果将为建立非等温烧结模型和烧结工艺参数的优化方法提供依据.