日期:
2020 年 — 2026 年
2020
2021
2022
2023
2024
2025
2026
影响因子:

Proteotoxic stress triggers TFEB- and TFE3-mediated autophagy and lysosomal biogenesis via non-canonical MTORC1 inactivation.

蛋白毒性应激通过非经典的 MTORC1 失活触发 TFEB 和 TFE3 介导的自噬和溶酶体生物合成。

Zhu Zhou, Yang Jing, Montefusco Sandro, Xia Siyu, Ou Jinhuan, Tong Haibo, Zeng Qingzhong, Xu Fengmei, Dai Lingyun, Sun Jichao, Xu Chengchao, Medina Diego Luis, Wang Jigang, Zhang Wei, Yang Chuanbin

GMPPB-CDG Results in Lysosomal Dysfunction and Acid Alpha-Glucosidase Deficiency.

GMPPB-CDG 导致溶酶体功能障碍和酸性α-葡萄糖苷酶缺乏。

Damiano Carla, Tarallo Antonietta, Gragnaniello Vincenza, Strollo Sandra, Fecarotta Simona, Tuzzi M Rosaria, Polishchuk Elena, Montefusco Sandro, Valanzano Anna, Assunto Antonia, Minopoli Nadia, Casa Roberto Della, Polishchuk Roman, Groen Stijn L M In 't, Medina Diego Luis, Bertini Enrico, Carrozzo Rosalba, Emmerich Julia, Schoser Benedikt, Pijnappel W W M Pim, Parenti Giancarlo

Drug-Induced Reversible Lysosomal Changes Tracked in Live Cells by Holo-Tomographic Flow Cytometry.

利用全息断层扫描流式细胞术追踪活细胞中药物诱导的可逆溶酶体变化

Pirone Daniele, Schiavo Michela, Giugliano Giusy, Montefusco Sandro, Miccio Lisa, Memmolo Pasquale, Medina Diego Luis, Ferraro Pietro

TRPML-1 Dysfunction and Renal Tubulopathy in Mucolipidosis Type IV.

TRPML-1 功能障碍与 IV 型粘脂病肾小管病变

Grieco Giuseppina, Montefusco Sandro, Nusco Edoardo, Capuozzo Antonella, Cervellini Francesca, Polishchuk Elena, Bishop Martha, Miele Antonio, D'Apolito Luciano, La Vecchia Claudia, Aurilia Miriam, Schiavo Michela, Staiano Leopoldo, Cesana Marcella, Oberman Rebecca, Lynch Anna V, Musolino Patricia, Trepiccione Francesco, Grishchuk Yulia, Medina Diego Luis

TPC2 rescues lysosomal storage in mucolipidosis type IV, Niemann-Pick type C1, and Batten disease

TPC2 可挽救 IV 型粘脂病、C1 型尼曼-匹克病和巴顿病中的溶酶体贮积。

Scotto Rosato, Anna; Krogsaeter, Einar K; Jaślan, Dawid; Abrahamian, Carla; Montefusco, Sandro; Soldati, Chiara; Spix, Barbara; Pizzo, Maria Teresa; Grieco, Giuseppina; Böck, Julia; Wyatt, Amanda; Wünkhaus, Daniela; Passon, Marcel; Stieglitz, Marc; Keller, Marco; Hermey, Guido; Markmann, Sandra; Gruber-Schoffnegger, Doris; Cotman, Susan; Johannes, Ludger; Crusius, Dennis; Boehm, Ulrich; Wahl-Schott, Christian; Biel, Martin; Bracher, Franz; De Leonibus, Elvira; Polishchuk, Elena; Medina, Diego L; Paquet, Dominik; Grimm, Christian

Identification of p38 MAPK and JNK as new targets for correction of Wilson disease-causing ATP7B mutants

鉴定出 p38 MAPK 和 JNK 为纠正威尔逊病致病 ATP7B 突变体的新靶点

Chesi, Giancarlo; Hegde, Ramanath N; Iacobacci, Simona; Concilli, Mafalda; Parashuraman, Seetharaman; Festa, Beatrice Paola; Polishchuk, Elena V; Di Tullio, Giuseppe; Carissimo, Annamaria; Montefusco, Sandro; Canetti, Diana; Monti, Maria; Amoresano, Angela; Pucci, Piero; van de Sluis, Bart; Lutsenko, Svetlana; Luini, Alberto; Polishchuk, Roman S

A reverse-engineering approach to dissect post-translational modulators of transcription factor's activity from transcriptional data

利用逆向工程方法,从转录数据中解析转录因子活性的翻译后修饰因子。

Gambardella, Gennaro; Peluso, Ivana; Montefusco, Sandro; Bansal, Mukesh; Medina, Diego L; Lawrence, Neil; di Bernardo, Diego

Copper binds the carboxy-terminus of trefoil protein 1 (TFF1), favoring its homodimerization and motogenic activity

铜与三叶蛋白1 (TFF1) 的羧基末端结合,促进其同源二聚化和运动活性。

Tosco, Alessandra; Monti, Maria Chiara; Fontanella, Bianca; Montefusco, Sandro; D'Andrea, Luca; Ziaco, Barbara; Baldantoni, Daniela; Rio, Marie-Christine; Marzullo, Liberato

AAV-mediated tyrosinase gene transfer restores melanogenesis and retinal function in a model of oculo-cutaneous albinism type I (OCA1)

AAV介导的酪氨酸酶基因转移可恢复I型眼皮肤白化病(OCA1)模型中的黑色素生成和视网膜功能。

Gargiulo, Annagiusi; Bonetti, Ciro; Montefusco, Sandro; Neglia, Simona; Di Vicino, Umberto; Marrocco, Elena; Corte, Michele Della; Domenici, Luciano; Auricchio, Alberto; Surace, Enrico M