Titlebook: JIMD Reports, Volume 36; Eva Morava,Matthias Baumgartner,Verena Peters Book 2017 Society for the Study of Inborn Errors of Metabolism (SSI

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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

sed-rate

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

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貪婪性

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

Harbor

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

FLIT

Mette Borch N?rmark,Nanna Kjaer,Allan Meldgaard Lund

annexation

Jan Rasmussen,David M. Hougaard,Noreen Sandhu,Katrine Fj?llegaard,Poula R. Petersen,Ulrike Steuerwal

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GUILE

Esther van Dam,Anne Daly,Gineke Venema-Liefaard,Margreet van Rijn,Terry G. J. Derks,Patrick J. McKie