Search Results - "Salvioli, R."

Clinical, biochemical and molecular characterization of prosaposin deficiency by Motta, M., Tatti, M., Furlan, F., Celato, A., Di Fruscio, G., Polo, G., Manara, R., Nigro, V., Tartaglia, M., Burlina, A., Salvioli, R.

“…Prosaposin (PSAP) deficiency is an ultra‐rare, fatal infantile lysosomal storage disorder (LSD) caused by variants in the PSAP gene, with seven subjects…”
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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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Reduced cathepsins B and D cause impaired autophagic degradation that can be almost completely restored by overexpression of these two proteases in Sap C-deficient fibroblasts by TATTI, Massimo, MOTTA, Marialetizia, DI BARTOLOMEO, Sabrina, SCARPA, Susanna, CIANFANELLI, Valentina, CECCONI, Francesco, SALVIOLI, Rosa

“…Saposin (Sap) C deficiency, a rare variant form of Gaucher disease, is due to mutations in the Sap C coding region of the prosaposin (PSAP) gene. Sap C is…”
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Clinical, biochemical and molecular characterization of prosaposin deficiency: Two new cases of prosaposin deficiency by Motta, M., Tatti, M., Furlan, F., Celato, A., Di Fruscio, G., Polo, G., Manara, R., Nigro, V., Tartaglia, M., Burlina, A., Salvioli, R.

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The N370S (Asn super(370)-->Ser) mutation affects the capacity of glucosylceramidase to interact with anionic phospholipid-containing membranes and saposin C by Salvioli, Rosa, Tatti, Massimo, Scarpa, Susanna, Moavero, Sabrina Maria, Ciaffoni, Fiorella, Felicetti, Federica, Kaneski, Christine R, Brady, Roscoe O, Vaccaro, Anna Maria

“…The properties of the endolysosomal enzyme GCase (glucosylceramidase), carrying the most prevalent mutation observed in Gaucher patients, namely substitution…”
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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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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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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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Interaction of saposin D with membranes: effect of anionic phospholipids and sphingolipids by Ciaffoni, Fiorella, Tatti, Massimo, Salvioli, Rosa, Vaccaro, Anna Maria

“…Saposin (Sap) D is an endolysosomal protein that, together with three other similar proteins, Sap A, Sap B and Sap C, is involved in the degradation of…”
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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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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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Structural analysis of saposin C and B. Complete localization of disulfide bridges by Vaccaro, A.M. (Istituto Superiore di Sanita, Roma, Italy.), Salvioli, R, Barca, A, Tatti, M, Ciaffoni, F, Maras, B, Siciliano, R, Zappacosta, F, Amoresano, A, Pucci, P

“…Saposins A, B, C, and D are a group of homologous glycoproteins derived from a single precursor, prosaposin, and apparently involved in the stimulation of the…”
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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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Purification and properties of arylsulfatase C from human placenta by Vaccaro, A M, Salvioli, R, Muscillo, M, Renola, L

“…Arylsulfatase C (ASC) was purified about 1,000-fold from human placenta. The major steps in the procedure included chromatography on Con A-Sepharose and…”
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Factors affecting the binding of glucosylceramidase to its natural substrate dispersion by Vaccaro, A M, Tatti, M, Ciaffoni, F, Salvioli, R

“…This paper reports the results of ultracentrifugation experiments devised for investigating the interactions occurring in the conditions of the enzymatic assay…”
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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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Function of saposin C in the reconstitution of glucosylceramidase by phosphatidylserine liposomes by Vaccaro, Anna Maria, Tatti, Massimo, Ciaffoni, Fiorella, Salvioli, Rosa, Maras, Bruno, Barca, Alessandra

“…The function of saposin C (Sap C), a glucosylceramidase activator protein, in the enzyme stimulation by phosphatidylserine (PS) liposomes has been…”
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Structural and membrane‐binding properties of saposin D by Tatti, Massimo, Salvioli, Rosa, Ciaffoni, Fiorella, Pucci, Piero, Andolfo, Annapaola, Amoresano, Angela, Vaccaro, Anna Maria

“…Saposin D is generated together with three similar proteins, saposins A, B and C, from a common precursor, called prosaposin, in acidic organelles such as late…”
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Function of saposin C in the reconstitution of glycosylceramidase by phosphatidylserine liposomes by VACCARO, A. M, TATTI, M, CIAFFONI, F, SALVIOLI, R, MARAS, B, BARCA, A

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