Search results for "Pompe"
showing 10 items of 60 documents
Characterization of a proton pump from Acer pseudoplatanus cell microsomes
1985
Abstract An Acer pseudoplatanus cell microsomal fraction was enriched in ATPase by sedimentation through a sucrose cushion and treatment with Triton X-100. This activity, which reached 0.9 μmol P i min −1 mg −1 protein, was specific for ATP, slightly stimulated by K + , inhibited by orthovanadate and diethylstilbestrol, insensitive to oligomycin and azide, and had a K m - value of 0.51 mM for MgATP. ATP-dependent proton translocation was demonstrated by the ΔpH probe acridine orange. This activity had a optimum at pH 6.5, was substrate specific for ATP, and was strongly dependent on K + . Preparations of plasma membrane ATPase from A. pseudoplatanus cell culture thus posses biochemical prop…
Vacuolated PAS-Positive Lymphocytes on Blood Smear: An Easy Screening Tool and a Possible Biomarker for Monitoring Therapeutic Responses in Late Onse…
2018
Background: Primary aim was to investigate the diagnostic value of PAS-positive vacuolated lymphocytes on blood smear in Late Onset Pompe Disease (LOPD) patients and, secondly, to evaluate its potential utility in monitoring treatment effects.Methods: We examined blood smear of 26 LOPD patients. We evaluated 10 treated and 16 untreated LOPD patients. Among the latter group, 7 patients later initiated ERT and were tested again 6 months after start. Blood smear was also sampled from 82 controls and 19 patients with other muscle glycogenoses (MGSDs). PAS staining was used to evaluate: (1) presence of lymphocytes with glycogen-filled vacuoles, (2) quantification of vacuolated lymphocytes.Result…
Central nervous system involvement in late-onset Pompe disease: clues from neuroimaging and neuropsychological analysis
2018
Background and purpose Late-onset Pompe disease (LOPD) is a rare, multisystem disorder that is well established to mainly impair skeletal muscle function. Systematic studies exploring brain functions in LOPD are lacking. The aim of this study was to detect morphological and functional brain alterations as well as neuropsychological impairment in LOPD. Methods We studied 21 patients (10 male, mean age 49 ± 18.4 years) with defined diagnosis of LOPD, divided into two groups: one with pre-symptomatic hyperCKemia with no muscle weakness and the second with limb-girdle muscle weakness. All patients underwent 3T magnetic resonance imaging (MRI) to obtain morphological/angiographic evaluation as w…
Per una lettura socio-economica dei contesti abitativi: L’insula I del Quartiere ellenistico-romano di Agrigento. La domus I A – I B
2021
In linea con le ultime ricerche organizzate dal Parco Archeologico Regionale Valle dei Templi di Agrigento, il cui scopo è la conoscenza dell'antica città di Akragas/Agrigentum come sistema integrato e unitario, si è dedicata nuova attenzione alle forme dell'abitare, in particolare nell'area del cosiddetto "Quartiere ellenistico-romano". Quest'area fu scavata negli anni '50 e '60 del secolo scorso, ma è con la pubblicazione di E. De Miro, avvenuta solo nel 2009, che è stato possibile conoscere i risultati delle indagini. In questo contributo ci si vuole concentrare sull'isolato I del Quartiere, contraddistinto da due grandi abitazioni (I A/B e I E/F) che dominano l'area pubblica dell'agorà.…
New insights to assess the consolidation of stone materials used in built heritage: the case study of ancient graffiti (Tituli Picti) in the archaeol…
2020
AbstractTituli Picti are an ancient form of urban graffiti very common in the archaeological site of Pompeii (Naples, South—Italy). They are generally made of red pigments applied on walls of Campanian ignimbrite. This paper deals with a scientific investigation aimed to their conservation. This is a challenging task since it requires a multidisciplinary approach that includes restorers, archaeologists and conservation scientists. The study has provided suggestions on the proper way to conserve Tituli Picti over time. In the present work, several specimens of Campanian ignimbrite were painted with red earth pigment; lime and Arabic gum have been used as binders as well. Such painted stones …
Safety, tolerability, pharmacokinetics, pharmacodynamics, and exploratory efficacy of the novel enzyme replacement therapy avalglucosidase alfa (neoG…
2019
This multicenter/multinational, open-label, ascending-dose study (NCT01898364) evaluated safety, tolerability, pharmacokinetics, pharmacodynamics, and exploratory efficacy of repeat-dose avalglucosidase alfa (neoGAA), a second-generation, recombinant acid α-glucosidase replacement therapy, in late-onset Pompe disease (LOPD). Patients ≥18 years, alglucosidase alfa naïve (Naïve) or previously receiving alglucosidase alfa for ≥9 months (Switch), with baseline FVC ≥50% predicted and independently ambulatory, received every-other-week avalglucosidase alfa 5, 10, or 20 mg/kg over 24 weeks. 9/10 Naïve and 12/14 Switch patients completed the study. Avalglucosidase alfa was well-tolerated; no deaths…
Las esculturas encargadas por Carlos V a Leone Leoni en 1549 y su acabado en España por Pompeo Leoni
2013
In Brussels in 1549 Charles V commissioned four bronze and four marble sculptures from Leone Leoni, to which were added new orders. The sculptor worked on them, especially the bronzes, in Milan, and they were shipped to Spain along with other works at the time of the arrival of the Emperor and the Court. This article discusses the history and evolution of the marbles and a bust (Prado Museum: E-260, E-262, E-267, E-269, E-291), which were sent from Genoa to Cartagena, where they remained until 1568. After years of neglect, Pompeo Leoni was commissioned to arrange for their transfer to his house in Madrid along with Juan de Lugano, artist and dealer in marbles, where Pompeo finished most of …
Allearsi col nemico. Pompeo, alcuni pregiudizi etnografici, i destini della storia nell’ottavo libro della Pharsalia di Lucano
2022
Analisi del passo dell'ottavo libro della Pharsalia, in cui si delibera sulle decisioni da prendere in merito alle alleanze. Il discorso si rivela carico di alcuni pregiudizi etnografici Analysis of the passage from the eighth book of Pharsalia, in which it deliberates on the decisions to be taken regarding the alliances. The speech turns out to be laden with some ethnographic prejudices
A new approximation procedure for fractals
2003
AbstractThis paper is based upon Hutchinson's theory of generating fractals as fixed points of a finite set of contractions, when considering this finite set of contractions as a contractive set-valued map.We approximate the fractal using some preselected parameters and we obtain formulae describing the “distance” between the “exact fractal” and the “approximate fractal” in terms of the preselected parameters. Some examples and also computation programs are given, showing how our procedure works.