crockery 发表于 2025-3-25 05:10:56

Widespread Expression of a Membrane-Tethered Version of the Soluble Lysosomal Enzyme Palmitoyl Protbrane-tethered PPT1 was then generated. We show that expression of lysosome-restricted PPT1 in vivo largely rescues the INCL biochemical, histological, and functional phenotype. These data suggest that lysosomal tethering of PPT1 via the C-terminus of LAMP1 is a viable strategy and that this general

混沌 发表于 2025-3-25 08:19:41

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sed-rate 发表于 2025-3-25 11:40:16

Altered Cellular Homeostasis in Murine MPS I Fibroblasts: Evidence of Cell-Specific Physiopathology Ca. and mitochondrial membrane potential and higher cytoplasmatic pH when compared to .+/+ animals. Also, Idua−/− fibroblasts were more resistant to the apoptotic induction with staurosporine, indicating a possible resistance to apoptotic induction in those cells. In addition, despite the intracell

好开玩笑 发表于 2025-3-25 19:34:56

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贪婪性 发表于 2025-3-25 20:31:55

Saskia Koene,Ilse Dirks,Esmee van Mierlo,Pascal R. de Vries,Anjo J. W. M. Janssen,Jan A. M. Smeitink

Harbor 发表于 2025-3-26 00:34:45

Alberto Benussi,Maria Sofia Cotelli,Maura Cosseddu,Valeria Bertasi,Marinella Turla,Ettore Salsano,An

FLIT 发表于 2025-3-26 05:14:05

Mette Borch Nørmark,Nanna Kjaer,Allan Meldgaard Lund

annexation 发表于 2025-3-26 12:06:09

Jan Rasmussen,David M. Hougaard,Noreen Sandhu,Katrine Fjællegaard,Poula R. Petersen,Ulrike Steuerwal

积云 发表于 2025-3-26 16:22:25

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GUILE 发表于 2025-3-26 16:51:49

Esther van Dam,Anne Daly,Gineke Venema-Liefaard,Margreet van Rijn,Terry G. J. Derks,Patrick J. McKie
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查看完整版本: Titlebook: JIMD Reports, Volume 36; Eva Morava,Matthias Baumgartner,Verena Peters Book 2017 Society for the Study of Inborn Errors of Metabolism (SSI