702 research outputs found
Nuovi clienti in nuovi mercati: il vino italiano in India
Il commercio internazionale \ue8 nel corso degli anni divenuto sempre pi\uf9 indispensabile per i grandi paesi produttori di vino, che vedono ormai nell\u2019export la possibilit\ue0 di vendere una quota della loro produzione che tendenzialmente supera la domanda interna, la quale invece appare in calo nei paesi con consolidata tradizione produttiva. I paesi di maggior interesse risultano essere ovviamente paesi ad elevato PIL pro capite, in fase di crescita economica e demograficamente rilevanti, tali da rappresentare potenziali mercati per lo sviluppo dell\u2019export. Tra questi vanno segnalati i cosiddetti BRIC, ovvero Brasile, Russia, India e Cina, che presentano alcuni tratti comuni, ma ovviamente anche problematiche specifiche legate alle peculiarit\ue0 del singolo paese o anche di aree sub-nazionali, vista la loro rilevante eterogeneit\ue0 socio-economica e culturale interna. Gli elementi pocanzi indicati evidenziano per\uf2 la necessit\ue0 per i produttori vinicoli di elaborare una strategia competitiva innovativa che possa permettere di penetrare efficacemente questi nuovi mercati; in tale senso appare necessario ricorrere ad approcci innovativi di marketing del vino. Per i produttori italiani di vino l\u2019individuazione di nuovi clienti in nuovi mercati geografici pu\uf2 rappresentare un interessante percorso di innovazione e sviluppo aziendale. In questa prospettiva, nel presente capitolo si analizzano le potenzialit\ue0 e le criticit\ue0 del mercato indiano del vino, un mercato ancora giovane che, pur di fronte a diversi ostacoli di tipo culturale, religioso, legislativo e tariffario, sta manifestando segnali, a volte contraddittori, di interesse e di crescita. Il presente capitolo ha l\u2019obiettivo di esplorare le prospettive e le criticit\ue0 del vino italiano in un mercato emergente come quello indiano che, pur presentando
tratti di grande interesse per gli operatori, \ue8 ancora caratterizzato da notevole incertezza sulle potenzialit\ue0 di sviluppo. Dal punto di vista metodologico, dopo un sintetico inquadramento del mercato, basato sull\u2019analisi di dati secondari e dei recenti contributi accademici9, si propongono i risultati di una ricerca qualitativa, realizzata, con finalit\ue0 esplorative attraverso interviste a diversi operatori
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Cataract and optic disk drusen in a patient with glycogenosis and di George syndrome: clinical and molecular report
Background
We report the ophthalmic findings of a patient with type Ia glycogen storage disease (GSD Ia), DiGeorge syndrome (DGS), cataract and optic nerve head drusen (ONHD).
Case presentation
A 26-year-old white woman, born at term by natural delivery presented with a post-natal diagnosis of GSD Ia. Genetic testing by array-comparative genomic hybridization (CGH) for DGS was required because of her low levels of serum calcium. The patient has been followed from birth, attending the day-hospital every six months at the San Paolo Hospital, Milan, outpatient clinic for metabolic diseases and previously at another eye center. During the last day-hospital visit, a complete eye examination showed ONHD and cataract in both eyes. Next Generation Sequencing (NGS) was subsequently done to check for any association between the eye problems and metabolic aspects.
Conclusions
This is the first description of ocular changes in a patient with GSD Ia and DGS. Mutations explaining GSD Ia and DGS were found but no specific causative mutation for cataract and ONHD. The metabolic etiology of her lens changes is known, whereas the pathogenesis of ONHD is not clear. Although the presence of cataract and ONHD could be a coincidence; the case reported could suggest that hypocalcemia due to DGS could be the common biochemical pathway
Design and Field Measurements of a Linear Accelerator Endowed with Single Feed with Movable Short Coupler
Field asymmetries in the rf coupler of accelerating structures degrade the projected beam transverse emittance, especially at low energy. This paper presents an alternative single feed coupler design that reduces the dipolar
and the quadrupolar field components by exploiting a movable short circuit placed on the opposite waveguide. The structure has been simulated and optimized with the Ansys HFSS simulation code. RF measurements on an aluminum prototype machined in the "Elettra - Sincrotrone Trieste S.C.p.A.", are here presented. Such results are in good agreement with the simulations
impact of non gaussian electron energy heating upon the performance of a seeded free electron laser
E. Ferrari, E. Allaria, W. Fawley, L. Giannessi, Z. Huang, G. Penco, and S. Spampinati Elettra-Sincrotrone Trieste S.C.p.A. di interesse nazionale, Strada Statale 14-km 163,5 in AREA Science Park, 34149 Basovizza, Trieste, Italy Universita degli Studi di Trieste, Dipartimento di Fisica, Piazzale Europa 1, 34127 Trieste, Italy SLAC National Accelerator Laboratory, Menlo Park, California 94025, USA Enea, via Enrico Fermi 45, 00044 Frascati, Roma, Italy Laboratory of Quantum Optics, University of Nova Gorica, 5000 Nova Gorica, Slovenia Department of Physics, University of Liverpool, Oxford Street L69 7ZE, Liverpool, United Kingdom Cockcroft Institute, Sci-Tech Daresbury, Keckwick Lane WA4 4AD, Daresbury, Warrington, United Kingdom (Received 11 October 2013; published 21 March 2014
Experimental demonstration of enhanced self-amplified spontaneous emission by an optical klystron.
We report the first experimental evidence of enhancement of self-amplified spontaneous emission, due to the use of an optical klystron. In this free-electron laser scheme, a relativistic electron beam passes through two undulators, separated by a dispersive section. The latter converts the electron-beam energy modulation produced in the first undulator in density modulation, thus enhancing the free-electron laser gain. The experiment has been carried out at the FERMI facility in Trieste. Powerful radiation has been produced in the extreme ultraviolet range, with an intensity a few orders of magnitude larger than in pure self-amplified spontaneous emission mode. Data have been benchmarked with an existing theoretical model. © 2015 American Physical Society
Two-colour generation in a chirped seeded Free-Electron Laser
We present the experimental demonstration of a method for generating two
spectrally and temporally separated pulses by an externally seeded, single-pass
free-electron laser operating in the extreme-ultraviolet spectral range. Our
results, collected on the FERMI@Elettra facility and confirmed by numerical
simulations, demonstrate the possibility of controlling both the spectral and
temporal features of the generated pulses. A free-electron laser operated in
this mode becomes a suitable light source for jitter-free, two-colour
pump-probe experiments
Genetic pre-participation screening in selected athletes: a new tool for the prevention of sudden cardiac death?
Sudden cardiac death (SCD) of athletes is a
topical issue. “Borderline cardiac abnormalities”, which occur in
~2% of elite male athletes, may result in SCD, which may have
a genetic base. Genetic analysis may help identify pathological
cardiac abnormalities. We performed phenotype-guided
genetic analysis in athletes who, pre-participation, showed
ECG and/or echo “borderline” abnormalities, to discriminate
subjects at a greater risk of SCD.
Methods: We studied 24 elite athletes referred by the National
Federation of Olympic sports; and 25 subjects seeking eligibility
to practice agonistic sport referred by the Osservatorio
Epidemiologico della Medicina dello Sport della Regione
Campania. Inclusion criteria: a) ECG repolarization borderline
abnormalities; b) benign ventricular arrhythmias; c) left
ventricular wall thickness in the grey zone of physiology versus
pathology (max wall thickness 12-15 mm in females; 13-16 mm
in males). Based on the suspected phenotype, we screened
subjects for the LMNA gene, for 8 sarcomeric genes, 5
desmosomal genes, and cardiac calcium, sodium and
potassium channel disease genes.
Results: Genetic analysis was completed in 37/49 athletes, 22
competitive and 27 non-competitive athletes, showing
“borderline” clinical markers suggestive of hypertrophic
cardiomyopathy (HCM,n. 24), dilated cardiomyopathy (n. 4),
arrhythmogenic right ventricular dysplasia/cathecholaminergic
polymorphic ventricular tachycardia (ARVD/CPVT, n. 11), long
QT syndrome (LQTS, n. 4), sick sinus syndrome (SSS, n. 5),
Brugada syndrome (BrS, n. 1). We identifyed 11 mutations in
9 athletes (an ARVD athlete was compound heterozygote for
the PKP2 gene and an HCM athlete was double heterozygote
for the MYBPC3 and TNNT2 genes): 3 known mutations
related to LQTS, HCM and ARVD, respectively, and 8 novel
mutations, located in the SCN5A, RyR2, PKP2, MYBPC3 and
ACTC1 genes. The new mutations were absent in ~800 normal
chromosomes and were predicted “probably damaging” by in
silico analysis. Patch clamp analysis in channelopathies
indicated for some mutation abnormal biophysical behavior of
the corresponding mutant protein.
Conclusion: Genetic analysis may help distinguish between
physiology and pathology in athletes with clinically suspected
heart disease
Full-length TDP-43 and its C-terminal domain form filamentsin vitrohaving non-amyloid properties
Accumulation of ubiquitin-positive, tau- and α-synuclein-negative intracellular inclusions of TDP-43 in the central nervous system represents the major hallmark correlated to amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U). Such inclusions have variably been described as amorphous aggregates or more structured deposits having amyloid properties. Here we have purified full-length TDP-43 (FL TDP-43) and its C-terminal domain (Ct TDP-43) to investigate the morphological, structural and tinctorial features of aggregates formed in vitro by them at pH 7.4 and 37 °C. AFM images indicate that both protein variants show a tendency to form filaments. Moreover, we show that both FL TDP-43 and Ct TDP-43 filaments possess a largely disordered secondary structure, as ascertained by far-UV circular dichroism and Fourier transform infra-red spectroscopy, do not bind Congo red and induce a very weak increase of thioflavin T fluorescence, indicating the absence of a clear amyloid-like signature
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