Search Results - "VACCARO, A. M"

Non-neuronopathic Gaucher disease due to saposin C deficiency by Tylki-Szymańska, A, Czartoryska, B, Vanier, M-T, Poorthuis, BJMH, Groener, JAE, Ługowska, A, Millat, G, Vaccaro, AM, Jurkiewicz, E

“…Gaucher disease is generally caused by a deficiency of the lysosomal enzyme glucocerebrosidase. The degradation of glycosphingolipids requires also the…”
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Biogenic habitat shifts under long-term ocean acidification show nonlinear community responses and unbalanced functions of associated invertebrates by Milazzo, M., Alessi, C., Quattrocchi, F., Chemello, R., D'Agostaro, R., Gil, J., Vaccaro, A.M., Mirto, S., Gristina, M., Badalamenti, F.

“…Experiments have shown that increasing dissolved CO2 concentrations (i.e. Ocean Acidification, OA) in marine ecosystems may act as nutrient for primary…”
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Saposins and their interaction with lipids by VACCARO, A. M, SALVIOLI, R, TATTI, M, CIAFFONI, F

“…The lysosomal degradation of several sphingolipids requires the presence of four small glycoproteins called saposins, generated by proteolytic processing of a…”
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Saposin C mutations in Gaucher disease patients resulting in lysosomal lipid accumulation, saposin C deficiency, but normal prosaposin processing and sorting by Vaccaro, Anna M., Motta, Marialetizia, Tatti, Massimo, Scarpa, Susanna, Masuelli, Laura, Bhat, Meenakshi, Vanier, Marie T., Tylki-Szymanska, Anna, Salvioli, Rosa

“…Gaucher disease (GD) is characterized by accumulation of glucosylceramide (GC) in the cells of monocyte/macrophage system. The degradation of GC is controlled…”
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First record of Pachygrapsus transversus (Gibbes, 1850) (Brachyura, Grapsidae) in Italian waters by Vaccaro, A. M., Pipitone, C.

“…Pachygrapsus transversus (Gibbes, 1850) is reported for the first time from Italian waters. This crab was collected at Ustica Island and at several other…”
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Peripheral neuropathy with hypomyelinating features in adult-onset Krabbe's disease by Sabatelli, M, Quaranta, L, Madia, F, Lippi, G, Conte, A, Lo Monaco, M, Di Trapani, G, Rafi, M.A, Wenger, D.A, Vaccaro, A.M, Tonali, P

“…We describe three brothers suffering from Krabbe's disease with onset in the fifth decade. The proband showed a complete deficiency of leukocyte enzyme…”
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Glucosylceramidase Mass and Subcellular Localization Are Modulated by Cholesterol in Niemann-Pick Disease Type C by Salvioli, Rosa, Scarpa, Susanna, Ciaffoni, Fiorella, Tatti, Massimo, Ramoni, Carlo, Vanier, Marie T., Vaccaro, Anna Maria

“…Niemann-Pick disease type C (NPC) is characterized by the accumulation of cholesterol and sphingolipids in the late endosomal/lysosomal compartment. The…”
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The binding of glucosylceramidase to glucosylceramide is promoted by its activator protein by Vaccaro, A.M., Muscillo, M., Salvioli, R., Tatti, M., Gallozzi, E., Suzuki, K.

“…A protein activator of glucosylceramidase (EC 3.2.1.45) has been previously identified by us in human placenta [(1985) Biochim. Biophys. Acta 836, 157–166]. In…”
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Saposin D Solubilizes Anionic Phospholipid-containing Membranes by Ciaffoni, F, Salvioli, R, Tatti, M, Arancia, G, Crateri, P, Vaccaro, A M

“…Saposin (Sap) D is a late endosomal/lysosomal small protein, generated together with three other similar proteins, Sap A, B, and C, from the common precursor,…”
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Correlation between the activity of glucosylceramidase and its binding to glucosylceramide-containing liposomes. Role of acidic phospholipids and fatty acids by Vaccaro, A M, Tatti, M, Salvioli, R, Ciaffoni, F, Gallozzi, E

“…Optimal enzymatic hydrolysis of glucosylceramide inserted into liposomes has been obtained when both acidic phospholipids and the appropriate fatty acids were…”
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Further studies on the reconstitution of glucosylceramidase activity by Sap C and anionic phospholipids by Salvioli, Rosa, Tatti, Massimo, Ciaffoni, Fiorella, Vaccaro, Anna Maria

“…The reconstitution of the activity of the lysosomal enzyme glucosylceramidase requires anionic phospholipids and, at least, a protein factor, saposin C (Sap…”
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pH-dependent Conformational Properties of Saposins and Their Interactions with Phospholipid Membranes (∗) by Vaccaro, Anna Maria, Ciaffoni, Fiorella, Tatti, Massimo, Salvioli, Rosa, Barca, Alessandra, Tognozzi, Deborah, Scerch, Chiara

“…Saposins A, B, C, and D are small lysosomal glycoproteins released by proteolysis from a single precursor polypeptide, prosaposin. We have presently…”
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Studies on glucosylceramidase binding to phosphatidylserine liposomes: the role of bilayer curvature by Vaccaro, A M, Tatti, M, Ciaffoni, F, Salvioli, R, Barca, A, Roncaioli, P

“…The influence of phosphatidylserine (PS) liposome size on their capacity to activate and bind purified glucosylceramidase was investigated. Gel filtration and…”
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Comparison of growth performance and biometric relationships in two reciprocal sturgeon hybrids reared in net cages (Sicily, Mediterranean) by Vaccaro, A.M, Buffa, G, Mirto, S, Sara, G, Mazzola, A

“…This study was designed to investigate growth performance in two sturgeon hybrids reared in two quadrangular floating cages moored in an artificial pond,…”
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Effect of Saposins A and C on the Enzymatic Hydrolysis of Liposomal Glucosylceramide by Vaccaro, Anna Maria, Tatti, Massimo, Ciaffoni, Fiorella, Salvioli, Rosa, Barca, Alessandra, Scerch, Chiara

“…The degradation of glucosylceramide in lysosomes is accomplished by glucosylceramidase with the assistance of, at least, another protein, saposin C (Sap C),…”
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Presence of activator proteins for the enzymatic degradation of glucosylceramide in several human tissues by Vaccaro, A M, Ciaffoni, F, Mandara, I, Suzuki, K

“…Glucosylceramidase (EC 3.2.1.45) protein activators, similar to the 'placental factor' previously identified by us in human placenta, have also been found in…”
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Reconstitution of glucosylceramidase on binding to acidic phospholipid-containing vesicles by Vaccaro, A M, Tatti, M, Ciaffoni, F, Salvioli, R, Roncaioli, P

“…Studies were conducted to investigate the mechanism by which acidic phospholipid-containing vesicles stimulate purified placental glucosylceramidase activity…”
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Importance of splicing for prosaposin sorting by Madar-Shapiro, L, Pasmanik-Chor, M, Vaccaro, A M, Dinur, T, Dagan, A, Gatt, S, Horowitz, M

“…The prosaposin gene encodes a 70 kDa protein. This protein might either reach the lysosomes and get processed there to four peptides, which are activators of…”
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Characterization of human glucosylsphingosine glucosyl hydrolase and comparison with glucosylceramidase by VACCARO, Anna Maria, MUSCILLO, Michele, SUZUKI, Kunihiko

“…Properties of glucosylsphingosine (gluco‐psychosine) glucosyl hydrolase were studied in detail in cultured human fibroblasts and placenta and were compared…”
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Saposin C induces pH-dependent destabilization and fusion of phosphatidylserine-containing vesicles by Vaccaro, Anna Maria, Tatti, Massimo, Ciaffoni, Fiorella, Salvioli, Rosa, Serafino, Annalucia, Barca, Alessandra

“…We have previously shown that saposin C (Sap C), a glucosylceramidase activator protein, interacts with phosphatidylserine (PS) large unilamellar vesicles…”
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