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

Allosteric Modulation of GCase Enhances Lysosomal Activity and Reduces ER Stress in GCase-Related Disorders.

GCase 的变构调节增强溶酶体活性并降低 GCase 相关疾病中的内质网应激

Fregno Ilaria, Pérez-Carmona Natalia, Rudinskiy Mikhail, Soldà Tatiana, Bergmann Timothy J, Ruano Ana, Delgado Aida, Cubero Elena, Bellotto Manolo, García-Collazo Ana María, Molinari Maurizio

A novel allosteric GCase modulator prevents Tau accumulation in GBA1(WT) and GBA1(L444P/L444P) cellular models.

一种新型变构 GCase 调节剂可防止 Tau 在 GBA1(WT) 和 GBA1(L444P/L444P) 细胞模型中积累

Ciccaldo Matteo, Pérez-Carmona Natàlia, Piovesana Ester, Cano-Crespo Sara, Ruano Ana, Delgado Aida, Fregno Ilaria, Calvo-Flores Guzmán Beatriz, Bellotto Manolo, Molinari Maurizio, Taylor Joanne, Papin Stéphanie, García-Collazo Ana María, Paganetti Paolo

CNX:FAM134B-driven ERLAD of ATZ polymers proceeds via enhanced formation of VAPA:ORP1L:RAB7 contact sites between ER and endolysosomes

CNX:FAM134B驱动的ATZ聚合物ERLAD是通过增强内质网和内溶酶体之间VAPA:ORP1L:RAB7接触位点的形成而进行的。

Fasana, Elisa; Fregno, Ilaria; Molinari, Maurizio

ER-to-lysosome-associated degradation acts as failsafe mechanism upon ERAD dysfunction

ER-溶酶体相关降解是 ERAD 功能障碍的安全机制

Elisa Fasana #, Ilaria Fregno #, Carmela Galli #, Tatiana Soldà, Maurizio Molinari

Validation of a highly sensitive HaloTag-based assay to evaluate the potency of a novel class of allosteric β-Galactosidase correctors

验证一种高灵敏度的基于 HaloTag 的检测方法,以评估新型变构 β-半乳糖苷酶校正剂的效力

Mikhail Rudinskiy, Maria Pons-Vizcarra, Tatiana Soldà, Ilaria Fregno, Timothy Jan Bergmann, Ana Ruano, Aida Delgado, Sara Morales, Xavier Barril, Manolo Bellotto, Elena Cubero, Ana María García-Collazo, Natalia Pérez-Carmona, Maurizio Molinari

N-glycan processing selects ERAD-resistant misfolded proteins for ER-to-lysosome-associated degradation

N-糖加工选择 ERAD 抗性错误折叠蛋白进行 ER 至溶酶体相关降解

Ilaria Fregno, Elisa Fasana, Tatiana Soldà, Carmela Galli, Maurizio Molinari

A selective ER-phagy exerts procollagen quality control via a Calnexin-FAM134B complex

选择性内质网自噬通过钙网蛋白-FAM134B复合物发挥前胶原蛋白质量控制作用。

Alison Forrester ,Chiara De Leonibus ,Paolo Grumati ,Elisa Fasana ,Marilina Piemontese ,Leopoldo Staiano ,Ilaria Fregno ,Andrea Raimondi ,Alessandro Marazza ,Gemma Bruno ,Maria Iavazzo ,Daniela Intartaglia ,Marta Seczynska ,Eelco van Anken ,Ivan Conte ,Maria Antonietta De Matteis ,Ivan Dikic ,Maurizio Molinari ,Carmine Settembre

Schwann cells ER-associated degradation contributes to myelin maintenance in adult nerves and limits demyelination in CMT1B mice

雪旺细胞内质网相关降解有助于维持成年神经中的髓鞘,并限制 CMT1B 小鼠的脱髓鞘。

Volpi, Vera G; Ferri, Cinzia; Fregno, Ilaria; Del Carro, Ubaldo; Bianchi, Francesca; Scapin, Cristina; Pettinato, Emanuela; Solda, Tatiana; Feltri, M Laura; Molinari, Maurizio; Wrabetz, Lawrence; D'Antonio, Maurizio

ER-to-lysosome-associated degradation of proteasome-resistant ATZ polymers occurs via receptor-mediated vesicular transport

内质网至溶酶体相关的蛋白酶体抗性 ATZ 聚合物的降解是通过受体介导的囊泡运输发生的

Ilaria Fregno, Elisa Fasana, Timothy J Bergmann, Andrea Raimondi, Marisa Loi, Tatiana Soldà, Carmela Galli, Rocco D'Antuono, Diego Morone, Alberto Danieli, Paolo Paganetti, Eelco van Anken, Maurizio Molinari

Eat it right: ER-phagy and recovER-phagy

正确食用:内质网自噬和恢复内质网自噬

Loi, Marisa; Fregno, Ilaria; Guerra, Concetta; Molinari, Maurizio