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

Deciphering α-L-Fucosidase Activity Contribution in Human and Mouse: Tissue α-L-Fucosidase FUCA1 Meets Plasma α-L-Fucosidase FUCA2.

解读 α-L-岩藻糖苷酶活性在人和小鼠中的作用:组织 α-L-岩藻糖苷酶 FUCA1 与血浆 α-L-岩藻糖苷酶 FUCA2 相遇。

Bäumges Hannah, Jelinek Svenja, Lange Heike, Markmann Sandra, Capriotti Emanuela, Häusser Jan Anwar, Ilse Mai-Britt, Braulke Thomas, Lübke Torben

The human disease gene LYSET is essential for lysosomal enzyme transport and viral infection

人类疾病基因LYSET对于溶酶体酶的运输和病毒感染至关重要。

Richards, Christopher M; Jabs, Sabrina; Qiao, Wenjie; Varanese, Lauren D; Schweizer, Michaela; Mosen, Peter R; Riley, Nicholas M; Klüssendorf, Malte; Zengel, James R; Flynn, Ryan A; Rustagi, Arjun; Widen, John C; Peters, Christine E; Ooi, Yaw Shin; Xie, Xuping; Shi, Pei-Yong; Bartenschlager, Ralf; Puschnik, Andreas S; Bogyo, Matthew; Bertozzi, Carolyn R; Blish, Catherine A; Winter, Dominic; Nagamine, Claude M; Braulke, Thomas; Carette, Jan E

Early enzyme replacement therapy prevents dental and craniofacial abnormalities in a mouse model of mucopolysaccharidosis type VI

早期酶替代疗法可预防粘多糖贮积症VI型小鼠模型出现牙齿和颅面畸形

Nagpal, Rohit; Georgi, Gina; Knauth, Sarah; Schmid-Herrmann, Carmen; Muschol, Nicole; Braulke, Thomas; Kahl-Nieke, Bärbel; Amling, Michael; Schinke, Thorsten; Koehne, Till; Petersen, Julian

Lysosomal proteome analysis reveals that CLN3-defective cells have multiple enzyme deficiencies associated with changes in intracellular trafficking.

溶酶体蛋白质组分析表明,CLN3 缺陷细胞存在多种酶缺陷,这些缺陷与细胞内运输的变化有关

Schmidtke Carolin, Tiede Stephan, Thelen Melanie, Käkelä Reijo, Jabs Sabrina, Makrypidi Georgia, Sylvester Marc, Schweizer Michaela, Braren Ingke, Brocke-Ahmadinejad Nahal, Cotman Susan L, Schulz Angela, Gieselmann Volkmar, Braulke Thomas

Quantitative Proteome Analysis of Mouse Liver Lysosomes Provides Evidence for Mannose 6-phosphate-independent Targeting Mechanisms of Acid Hydrolases in Mucolipidosis II

小鼠肝溶酶体的定量蛋白质组分析为粘脂病II中酸性水解酶的甘露糖-6-磷酸非依赖性靶向机制提供了证据

Markmann, Sandra; Krambeck, Svenja; Hughes, Christopher J; Mirzaian, Mina; Aerts, Johannes M F G; Saftig, Paul; Schweizer, Michaela; Vissers, Johannes P C; Braulke, Thomas; Damme, Markus

Single-chain antibody-fragment M6P-1 possesses a mannose 6-phosphate monosaccharide-specific binding pocket that distinguishes N-glycan phosphorylation in a branch-specific manner†

单链抗体片段 M6P-1 具有甘露糖-6-磷酸单糖特异性结合口袋,能够以分支特异性方式区分 N-聚糖磷酸化†

Blackler, Ryan J; Evans, Dylan W; Smith, David F; Cummings, Richard D; Brooks, Cory L; Braulke, Thomas; Liu, Xinyu; Evans, Stephen V; Müller-Loennies, Sven

Lysoplex: An efficient toolkit to detect DNA sequence variations in the autophagy-lysosomal pathway

Lysoplex:一种用于检测自噬-溶酶体途径中DNA序列变异的高效工具包

Di Fruscio, Giuseppina; Schulz, Angela; De Cegli, Rossella; Savarese, Marco; Mutarelli, Margherita; Parenti, Giancarlo; Banfi, Sandro; Braulke, Thomas; Nigro, Vincenzo; Ballabio, Andrea

Mannose 6 phosphorylation of lysosomal enzymes controls B cell functions

甘露糖6磷酸化作用调控溶酶体酶的功能

Otomo, Takanobu; Schweizer, Michaela; Kollmann, Katrin; Schumacher, Valéa; Muschol, Nicole; Tolosa, Eva; Mittrücker, Hans-Willi; Braulke, Thomas

Analyses of disease-related GNPTAB mutations define a novel GlcNAc-1-phosphotransferase interaction domain and an alternative site-1 protease cleavage site

对与疾病相关的GNPTAB突变的分析确定了一个新的GlcNAc-1-磷酸转移酶相互作用域和一个替代的位点1蛋白酶切割位点。

Velho, Renata Voltolini; De Pace, Raffaella; Klünder, Sarah; Sperb-Ludwig, Fernanda; Lourenço, Charles Marques; Schwartz, Ida V D; Braulke, Thomas; Pohl, Sandra

Molecular characterization of arylsulfatase G: expression, processing, glycosylation, transport, and activity.

芳基硫酸酯酶 G 的分子特征:表达、加工、糖基化、运输和活性

Kowalewski Björn, Lübke Torben, Kollmann Katrin, Braulke Thomas, Reinheckel Thomas, Dierks Thomas, Damme Markus