Search results for " LAX"

2018

The cutis laxa syndromes are multisystem disorders that share loose redundant inelastic and wrinkled skin as a common hallmark clinical feature. The underlying molecular defects are heterogeneous and 13 different genes have been involved until now, all of them being implicated in elastic fiber assembly. We provide here molecular and clinical characterization of three unrelated patients with a very rare phenotype associating cutis laxa, facial dysmorphism, severe growth retardation, hyperostotic skeletal dysplasia and intellectual disability. This disorder called Lenz-Majewski syndrome (LMS) is associated with gain of function mutations in PTDSS1, encoding an enzyme involved in phospholipid …

Extending the ALDH18A1 clinical spectrum to severe autosomal recessive fetal cutis laxa with corpus callosum agenesis

2018

IF 3.822 (2018); International audience

How preconditioning and pretensioning of grafts used in ACLigaments surgical reconstruction are influenced by their mechanical time-dependent charact…

2021

Abstract Purpose Consensus about a pre-implant preparation protocol adaptable to any graft used in Anterior Cruciate Ligament reconstruction is still lacking. In fact, there is not agreement on reliable metrics that consider both specific graft dimensional characteristics, such as its diameter, and the inherent properties of its constitutive material, i.e. ligaments or tendons. Aim of the present study was to investigate and propose the applied engineering stress as a possible metrics. Methods Preconditioning and pretensioning protocol involved groups of grafts with different section (10 or 32 mm2) and materials (i.e. human patellar and hamstring tendons, and synthetic grafts). A 140 N load…

Nomenclatural remarks on Carex sect. Sylvaticae (Cyperaceae): C. laxula and related names

2018

Our taxonomic and nomenclatural research reveals that the name ‘Carex laxula Tineo ex Boott’ (section Sylvaticae) has not been validly published. Consequently, the oldest available name for the species is Carex paui Sennen. A lectotype for the valid name Carex sylvatica var. laxula Tineo ex Guss. is here designated.

Typhetum laxmannii (Ubrizsy 1961) Nedelcu 1968 - The new plant association in Poland

2011

<em>Typhetum laxmannii</em> (Ubrizsy 1961) Nedelcu 1968 is a plant association new to Poland, built by an expansive kenophyte - <em>Typha laxmannii</em> Lepech. This paper presents the general distribution of both, the species and the association, paying particular attention to the area of Europe and Poland where, in recent years, many new locations as well as an increasing participation in vegetation cover have been observed. The habitat preferences of <em>Typhetum laxmannii</em>, the floristic composition of the association and its geographical differentiation within the occupied area are described. The current distribution of the association in Poland …

Two New Triterpene Saponins fromAcanthophyllum laxiusculum

2015

Two new triterpene glycosides, 1 and 2, together with three known ones, were isolated from roots of Acanthophyllum laxiusculum Schiman-Czeika. The structures of the new compounds were established by extensive 1D- and 2D-NMR spectroscopic experiments and MS analyses as 23-O-β-D-galactopyranosylgypsogenic acid 28-O-{β-D-glucopyranosyl-(1→2)-6-O-[4-carboxy-3-hydroxy-3-methyl-1-oxobutyl]-β-D-glucopyranosyl-(1→6)}-[β-D-glucopyranosyl-(1→3)]-β-D-galactopyranosyl ester (1) and gypsogenic acid 28-O-{β-D-glucopyranosyl-(1→2)-6-O-[4-carboxy-3-hydroxy-3-methyl-1-oxobutyl]-β-D-glucopyranosyl-(1→6)}-[β-D-glucopyranosyl-(1→3)]-β-D-galactopyranosyl ester (2).

Discrete KP Equation and Momentum Mapping of Toda System

2003

Abstract A new approach to discrete KP equation is considered, starting from the Gelfand-Zakhharevich theory for the research of Casimir function for Toda Poisson pencil. The link between the usual approach through the use of discrete Lax operators, is emphasized. We show that these two different formulations of the discrete KP equation are equivalent and they are different representations of the same equations. The relation between the two approaches to the KP equation is obtained by a change of frame in the space of upper truncated Laurent series and translated into the space of shift operators.

Biochemical, morphological and immunological findings in a patient with a cutis laxa-associated inborn disorder (De Barsy syndrome).

1986

Clinical symptoms of a male infant are described and compared with cases now classified as the De Barsy syndrome, a distinct disorder related to cutis laxa. Morphologically, clastic fibres in skin were frayed and reduced in number and density. The collagen fibril network was normal. Biochemical studies on collagen metabolism in a skin specimen and in cultured skin fibroblasts showed a normal amino acid content and a normal electrophoretic pattern of collagen constituents. The chemotactic migration of cultured fibroblasts was diminished when compared with fibroblasts from donors of different age groups. Immunological investigations revealed an impaired granulocyte function.

Formation of Variously Shaped Gold Nanoparticles by Anabaena laxa

2017

Anew aspect of this study is the biocatalysis ability of Anabaena laxa—fresh-water strain—which to date has not been studied with respect to the formation of gold nanoparticles. The results of our study have shown that A. laxa is able to form gold nanoparticles (AuNPs) at good yields within 24 h of incubation at all three tested concentrations (0.1, 0.5 and 1 mM) of hydrogen tetrachloroaurate (III). After 24 h, nanoparticles were mostly localized in the growth medium, which significantly facilitates their isolation. However, the elongation of this process is accompanied by their concentration in the exopolysaccharide (EPS) layer of the cyanobacterial cells. There is a correlation between th…

An order-adaptive compact approximation Taylor method for systems of conservation laws

2021

Abstract We present a new family of high-order shock-capturing finite difference numerical methods for systems of conservation laws. These methods, called Adaptive Compact Approximation Taylor (ACAT) schemes, use centered ( 2 p + 1 ) -point stencils, where p may take values in { 1 , 2 , … , P } according to a new family of smoothness indicators in the stencils. The methods are based on a combination of a robust first order scheme and the Compact Approximate Taylor (CAT) methods of order 2p-order, p = 1 , 2 , … , P so that they are first order accurate near discontinuities and have order 2p in smooth regions, where ( 2 p + 1 ) is the size of the biggest stencil in which large gradients are n…