G
Gianfranco Distefano
Researcher at Vita-Salute San Raffaele University
Publications - 10
Citations - 575
Gianfranco Distefano is an academic researcher from Vita-Salute San Raffaele University. The author has contributed to research in topics: PKD1 & Cilium. The author has an hindex of 8, co-authored 9 publications receiving 504 citations.
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Journal ArticleDOI
Polycystin-1 regulates extracellular signal-regulated kinase-dependent phosphorylation of tuberin to control cell size through mTOR and its downstream effectors S6K and 4EBP1.
Gianfranco Distefano,Manila Boca,Isaline Rowe,Claas Wodarczyk,Li Ma,Klaus Piontek,Gregory G. Germino,Pier Paolo Pandolfi,Alessandra Boletta +8 more
TL;DR: It is shown that PC-1 controls the mTOR pathway in a Tsc2-dependent manner, by inhibiting the extracellular signal-regulated kinase (ERK)-mediated phosphorylation of tuberin in Ser664.
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Polycystin-1 Induces Cell Migration by Regulating Phosphatidylinositol 3-kinase-dependent Cytoskeletal Rearrangements and GSK3β-dependent Cell–Cell Mechanical Adhesion
Manila Boca,Lisa D'Amato,Gianfranco Distefano,Roman S. Polishchuk,Gregory G. Germino,Alessandra Boletta +5 more
TL;DR: It is proposed that PC-1 might be a central regulator of epithelial plasticity and its loss results in impaired normal epithelial homeostasis.
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Polycystin-1 Induces Resistance to Apoptosis through the Phosphatidylinositol 3-Kinase/Akt Signaling Pathway
Manila Boca,Gianfranco Distefano,Feng Qian,Anil K. Bhunia,Gregory G. Germino,Alessandra Boletta +5 more
TL;DR: It is found that PC-1 expression results in phosphorylation of Akt and downstream effectors and that phosphatidylinositol 3-kinase (PI3-K) inhibitors prevent this process, and it is shown that dominant negative Akt can revertPC-1-induced protection from apoptosis.
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Polycystin-1 binds Par3/aPKC and controls convergent extension during renal tubular morphogenesis
Maddalena Castelli,Manila Boca,Marco Chiaravalli,Harini Ramalingam,Isaline Rowe,Gianfranco Distefano,Thomas L. Carroll,Alessandra Boletta +7 more
TL;DR: It is demonstrated that PC-1 plays an essential role in establishment of correct tubular diameter during nephron development and it regulates a program of cell polarity important for oriented cell migration and for a convergent extension-like process during tubular morphogenesis.
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Dissection of metabolic reprogramming in polycystic kidney disease reveals coordinated rewiring of bioenergetic pathways.
Christine Podrini,Isaline Rowe,Roberto Pagliarini,Ana S. H. Costa,Marco Chiaravalli,Ivano Di Meo,Hyunho Kim,Gianfranco Distefano,Valeria Tiranti,Feng Qian,Diego di Bernardo,Christian Frezza,Alessandra Boletta +12 more
TL;DR: It is found that Pkd1 loss leads to profound metabolic changes, including asparagine synthase-driven glutamine anaplerosis, and silencing of Asns is lethal in PkD1-mutant cells when combined with glucose deprivation, suggesting therapeutic approaches for ADPKD.