Edict de création en tiltre d'office formé de cent conseillers et secrétaires ordinaires de la chambre du Roy... [D'octobre 1613.]

[Acte royal. 1613-10-00]

A time-domain method for the analysis of thermal impedance response preserving the convolution form

The study of the thermal behavior of complex packages as multichip modules (MCM¿s) is usually carried out by measuring the so-called thermal impedance response, that is: the transient temperature after a power step. From the analysis of this signal, the thermal frequency response can be estimated, and consequently, compact thermal models may be extracted. We present a method to obtain an estimate of the time constant distribution underlying the observed transient. The method is based on an iterative deconvolution that produces an approximation to the time constant spectrum while preserving a convenient convolution form. This method is applied to the obtained thermal response of a microstructure as analyzed by finite element method as well as to the measured thermal response of a transistor array integrated circuit (IC) in a SMD package.

PLEKHG5 deficiency leads to an intermediate form of autosomal-recessive Charcot-Marie-Tooth disease.

Charcot-Marie-Tooth disease (CMT) comprises a clinically and genetically heterogeneous group of peripheral neuropathies characterized by progressive distal muscle weakness and atrophy, foot deformities and distal sensory loss. Following the analysis of two consanguineous families affected by a medium to late-onset recessive form of intermediate CMT, we identified overlapping regions of homozygosity on chromosome 1p36 with a combined maximum LOD score of 5.4. Molecular investigation of the genes from this region allowed identification of two homozygous mutations in PLEKHG5 that produce premature stop codons and are predicted to result in functional null alleles. Analysis of Plekhg5 in the mouse revealed that this gene is expressed in neurons and glial cells of the peripheral nervous system, and that knockout mice display reduced nerve conduction velocities that are comparable with those of affected individuals from both families. Interestingly, a homozygous PLEKHG5 missense mutation was previously reported in a recessive form of severe childhood onset lower motor neuron disease (LMND) leading to loss of the ability to walk and need for respiratory assistance. Together, these observations indicate that different mutations in PLEKHG5 lead to clinically diverse outcomes (intermediate CMT or LMND) affecting the function of neurons and glial cells.

Gauge group and reality conditions in Ashtekar's complex formulation of canonical gravity

We discuss reality conditions and the relation between spacetime diffeomorphisms and gauge transformations in Ashtekars complex formulation of general relativity. We produce a general theoretical framework for the stabilization algorithm for the reality conditions, which is different from Diracs method of stabilization of constraints. We solve the problem of the projectability of the diffeomorphism transformations from configuration-velocity space to phase space, linking them to the reality conditions. We construct the complete set of canonical generators of the gauge group in the phase space which includes all the gauge variables. This result proves that the canonical formalism has all the gauge structure of the Lagrangian theory, including the time diffeomorphisms.

A homozygous missense mutation in SCNN1A is responsible for a transient neonatal form of pseudohypoaldosteronism type 1.

Pseudohypoaldosteronism type 1 (PHA1) is a monogenic disorder of mineralocorticoid resistance characterized by salt wasting, hyperkalemia, high aldosterone levels, and failure to thrive. An autosomal recessive form (AR-PHA1) is caused by mutations in the epithelial sodium channel ENaC with usually severe and persisting multiorgan symptoms. The autosomal dominant form of PHA1 (AD-PHA1) is due to mutations in the mineralocorticoid receptor causing milder and transient symptoms restricted to the kidney. We identified a homozygous missense mutation in the SCNN1A gene (c.727T>C/p.Ser(243)Pro), encoding α-subunit of ENaC (α-ENaC) in a prematurely born boy with a severe salt-losing syndrome. The patient improved rapidly under treatment, and dietary salt supplementation could be stopped after 6 mo. Interestingly, the patient's sibling born at term and harboring the same homozygous Ser(243)Pro mutation showed no symptom of salt-losing nephropathy. In vitro expression of the αSer(243)Pro ENaC mutant revealed a slight but significant decrease in ENaC activity that is exacerbated in the presence of high Na(+) load. Our study provides the first evidence that ENaC activity is critical for the maintenance of salt balance in the immature kidney of preterm babies. Together with previous studies, it shows that, when the kidney is fully mature, the severity of the symptoms of AR-PHA1 is related to the degree of the ENaC loss of function. Finally, this study identifies a novel functional domain in the extracellular loop of ENaC.

Peripheral neuropathy is linked to a severe form of myotonic dystrophy in transgenic mice.

Myotonic dystrophy type 1 (DM1) is a multisystem disorder with a variable phenotype. The involvement of peripheral nerves in DM1 disease is controversial. The DM1 animal model DM300 transgenic mice that carry 350 to 500 CTG repeats express a mild DM1 phenotype but do not exhibit motor or sensory pathology. Here, we investigated the presence or absence of peripheral neuropathy in transgenic mice (DMSXL) that carry more than 1,300 CTG repeats and display a severe form of DM1. Electrophysiologic, histologic, and morphometric methods were used to investigate the structure and function of peripheral nerves. We observed lower compound muscle action potentials recorded from hind limb muscles and slowing of sciatic nerve conduction velocity in DMSXL versus control mice. Morphometric analyses showed an axonopathy and neuronopathy in the DMSXL mice characterized by a decrease in numbers of myelinatedmotor axons in sciatic nerve and in spinal cord motor neurons. Pathologic alterations in the structure of hind limb neuromuscular junctions were also detected in the DMSXL mice. These results suggest that peripheral neuropathy can be linked to a large CTG expansion and a severe form of DM1.

Canonical Noether symmetries and commutativity properties for gauge systems

For a dynamical system defined by a singular Lagrangian, canonical Noether symmetries are characterized in terms of their commutation relations with the evolution operators of Lagrangian and Hamiltonian formalisms. Separate characterizations are given in phase space, in velocity space, and through an evolution operator that links both spaces. 2000 American Institute of Physics.

Lee HWA Chung theorem for presympectic manifolds. Canonical transformations for constrained systems

We generalize the analogous of Lee Hwa Chungs theorem to the case of presymplectic manifolds. As an application, we study the canonical transformations of a canonical system (M, S, O). The role of Dirac brackets as a test of canonicity is clarified.

Canonical transformations theory for presymplectic systems

We develop a theory of canonical transformations for presymplectic systems, reducing this concept to that of canonical transformations for regular coisotropic canonical systems. In this way we can also link these with the usual canonical transformations for the symplectic reduced phase space. Furthermore, the concept of a generating function arises in a natural way as well as that of gauge group.

Poincar-Cartan intregral invariant and canonical trasformation for singular Lagrangians: an addendum

The results of a previous work, concerning a method for performing the canonical formalism for constrained systems, are extended when the canonical transformation proposed in that paper is explicitly time dependent.

Alpha-band suppression in the visual word form area as a functional bottleneck to consciousness.

The current state of empirical investigations refers to consciousness as an all-or-none phenomenon. However, a recent theoretical account opens up this perspective by proposing a partial level (between nil and full) of conscious perception. In the well-studied case of single-word reading, short-lived exposure can trigger incomplete word-form recognition wherein letters fall short of forming a whole word in one's conscious perception thereby hindering word-meaning access and report. Hence, the processing from incomplete to complete word-form recognition straightforwardly mirrors a transition from partial to full-blown consciousness. We therefore hypothesized that this putative functional bottleneck to consciousness (i.e. the perceptual boundary between partial and full conscious perception) would emerge at a major key hub region for word-form recognition during reading, namely the left occipito-temporal junction. We applied a real-time staircase procedure and titrated subjective reports at the threshold between partial (letters) and full (whole word) conscious perception. This experimental approach allowed us to collect trials with identical physical stimulation, yet reflecting distinct perceptual experience levels. Oscillatory brain activity was monitored with magnetoencephalography and revealed that the transition from partial-to-full word-form perception was accompanied by alpha-band (7-11 Hz) power suppression in the posterior left occipito-temporal cortex. This modulation of rhythmic activity extended anteriorly towards the visual word form area (VWFA), a region whose selectivity for word-forms in perception is highly debated. The current findings provide electrophysiological evidence for a functional bottleneck to consciousness thereby empirically instantiating a recently proposed partial perspective on consciousness. Moreover, the findings provide an entirely new outlook on the functioning of the VWFA as a late bottleneck to full-blown conscious word-form perception.

Heb 5.7, Jesus' Prayer on the Mount of Olives and Jewish Christianity: Hearing Early Christian Voices in Canonical and Apocryphal Texts

(Résumé de l'ouvrage) The book of Hebrews has often been the Cinderella of the New Testament, overlooked and marginalized; and yet it is one of the most interesting and theologically significant books in the New Testament. A Cloud of Witness examines the theology of the book in the light of its ancient historical context. There are chapters devoted to the structure of Hebrews, the person of Jesus Christ, Hebrews within the context of Second Temple Judaism and the Greco-Roman empire and the role of Hebrews in early Christian thought.