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1.
Franois Ancien Fabrizio Pucci Marianne Rooman 《International journal of molecular sciences》2021,22(9)
Sphingomyelin phosphodiesterase (SMPD1) is a key enzyme in the sphingolipid metabolism. Genetic SMPD1 variants have been related to the Niemann-Pick lysosomal storage disorder, which has different degrees of phenotypic severity ranging from severe symptomatology involving the central nervous system (type A) to milder ones (type B). They have also been linked to neurodegenerative disorders such as Parkinson and Alzheimer. In this paper, we leveraged structural, evolutionary and stability information on SMPD1 to predict and analyze the impact of variants at the molecular level. We developed the SMPD1-ZooM algorithm, which is able to predict with good accuracy whether variants cause Niemann-Pick disease and its phenotypic severity; the predictor is freely available for download. We performed a large-scale analysis of all possible SMPD1 variants, which led us to identify protein regions that are either robust or fragile with respect to amino acid variations, and show the importance of aromatic-involving interactions in SMPD1 function and stability. Our study also revealed a good correlation between SMPD1-ZooM scores and in vitro loss of SMPD1 activity. The understanding of the molecular effects of SMPD1 variants is of crucial importance to improve genetic screening of SMPD1-related disorders and to develop personalized treatments that restore SMPD1 functionality. 相似文献
2.
A Poblet S Andrade M Scagliola C Vodopivez A Curtosi A Pucci J Marcovecchio 《Canadian Metallurgical Quarterly》1997,207(2-3):187-194
Trace-metal contents were recorded for the epilithic antarctic lichens Usnea aurantiacoatra and U. antartica, sampled close to the Argentine scientific station 'Jubany' on '25 de Mayo' (King George) Island, in the Southern Shetland Archipelago (Antarctica). The corresponding heavy-metal levels have been measured through atomic absorption spectrophotometry, following internationally accepted analytical methods. The results obtained support the hypothesis that an atmospheric circulation of trace metals exists on the assessed area, and the activities developed at the different scientific stations located on this island would be a potential source of heavy metals to the evaluated environment. The geographical distribution of trace metals atmospherically transported in the area close to 'Jubany Station' was studied through the corresponding metal contents of the assessed lichens. Finally, the suitability of both analyzed lichen species, Usnea aurantiacoatra and U. antartica, as biological indicators for quantitative monitoring of airborne metals for this antarctic environment was recognized. 相似文献
3.
The Alder ene functionalization reaction of double bonds containing macromolecules such as polyisobutene oligomer (PIB) and a styrene-butadiene-styrene triblock copolymer (SBS) samples with maleic anhydride (MAH) or diethyl maleate (DEM) as enophiles is described. The analysis of the products by means of different techniques assesses the addition of the polar molecules to the reactive vinylidene units of the polymer with functional degrees (FD) depending on the type of enophile and polymer reactivity. The role of the reaction conditions and the use as Lewis acids as catalysts are discussed in terms of their influence on the addition reaction extent and on the polymer molecular weight. 相似文献
4.
We study the complexity of routing a set of messages with multiple destinations (multicast routing) on an n-node square mesh under the store-and-forward model. A standard argument proves that
time is required to route n messages, where each message is generated by a distinct node and at most c messages are to be delivered to any individual node. The obvious approach of simply replicating each message into the appropriate
number of unicast (single-destination) messages and routing these independently does not yield an optimal algorithm. We provide
both randomized and deterministic algorithms for multicast routing, which use constant-size buffers at each node. The randomized
algorithm attains optimal performance, while the deterministic algorithm is slower by a factor of O( log 2
n). We also describe an optimal deterministic algorithm that, however, requires large buffers of size O(c).
A preliminary version of this paper appeared in Proceedings of the 13th Annual ACM Symposium on Parallel Algorithms and Architectures,
Crete, Greece, 2001. This work was supported, in part, by MIUR under project ALGO-NEXT. 相似文献
5.
Diana Nanova Sebastian Beck Andreas Fuchs Tobias Glaser Christian Lennartz Wolfgang Kowalsky Annemarie Pucci Michael Kroeger 《Organic Electronics》2012,13(7):1237-1244
The degree of charge transfer in thin films of organic charge transfer (CT)-complexes, which are deposited via thermal evaporation, is examined via infrared-spectroscopy. We demonstrate a linear relationship between the shift in the excitation energy of the CN-stretching mode of CT-complexes with the acceptor 7,7,8,8-tetracyanoquinodimethane (TCNQ) and the charge transfer. The measured correlation corresponds very well with DFT calculations. For Na-TCNQ we observe a splitting in the peak of the CN-stretching mode, which can be explained by the coupling of two modes and was confirmed by the calculations. In CT-complexes with partial charge transfer the appearance of an electronic excitation is demonstrated. 相似文献
6.
7.
A new adaptive integration methodology for estimating flux in induction machine drives 总被引:6,自引:0,他引:6
Cirrincione M. Pucci M. Cirrincione G. Capolino G.-A. 《Power Electronics, IEEE Transactions on》2004,19(1):25-34
This paper proposes a simple adaptive notch filter for the elimination of the dc component in the integration of signals used for the flux estimation in high performance ac drives. This integration method is composed of two identical adaptive noise cancellers using a linear neural network with just one bias weight. Its behavior has been investigated in simulation as applied to electrical drives and compared with other four traditional integration algorithms. A test bench has been then developed for its experimentation in a field oriented controlled induction machine. It has been verified that this integration algorithm outperforms the other algorithms in estimating the rotor flux even at low speeds. 相似文献
8.
Effect of white wheat bread and white wheat bread added with bioactive compounds on hypercholesterolemic and steatotic mice fed a high‐fat diet
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9.
10.
We investigated the optical properties and the degradation process of the organic p-type semiconductor poly(triarylamine) (PTAA) in the mid infrared region. The dielectric function of PTAA was determined in the spectral region of 700 up to 6000 cm−1 by modeling ellipsometric measurements. Due to degradation at 65 °C, PTAA thin films developed carbonyl and hydroxyl features in the infrared spectra. Degradation under 85% RH additionally led to absorption signals of water. A degradation of the bare gold substrates was also observed. For bare gold films, morphology changed upon degradation and adsorption of hydrocarbons from the ambience took place. 相似文献