Search Results - "van der Lienden, Martijn J.C."

Glycosphingolipids and lysosomal storage disorders as illustrated by gaucher disease by Aerts, Johannes M.F.G., Kuo, Chi-Lin, Lelieveld, Lindsey T., Boer, Daphne E.C., van der Lienden, Martijn J.C., Overkleeft, Herman S., Artola, Marta

“…Glycosphingolipids are important building blocks of the outer leaflet of the cell membrane. They are continuously recycled, involving fragmentation inside…”
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Glycoprotein Non-Metastatic Protein B: An Emerging Biomarker for Lysosomal Dysfunction in Macrophages by van der Lienden, Martijn J C, Gaspar, Paulo, Boot, Rolf, Aerts, Johannes M F G, van Eijk, Marco

“…Several diseases are caused by inherited defects in lysosomes, the so-called lysosomal storage disorders (LSDs). In some of these LSDs, tissue macrophages…”
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Glycoprotein non-metastatic protein B (GPNMB) plasma values in patients with chronic visceral acid sphingomyelinase deficiency by Eskes, Eline C.B., van der Lienden, Martijn J.C., Sjouke, Barbara, van Vliet, Laura, Brands, Marion M.M.G., Hollak, Carla E.M., Aerts, Johannes M.F.G.

“…Acid sphingomyelinase deficiency (ASMD) is a rare LSD characterized by lysosomal accumulation of sphingomyelin, primarily in macrophages. With the recent…”
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HEPES‐buffering of bicarbonate‐containing culture medium perturbs lysosomal glucocerebrosidase activity by Lienden, Martijn J. C., Aten, Jan, Boot, Rolf G., Eijk, Marco, Aerts, Johannes M. F. G., Kuo, Chi‐Lin

“…Glucocerebrosidase (GCase), encoded by the GBA gene, degrades the ubiquitous glycosphingolipid glucosylceramide. Inherited GCase deficiency causes Gaucher…”
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Localization of active endogenous and exogenous β‐glucocerebrosidase by correlative light‐electron microscopy in human fibroblasts by van Meel, Eline, Bos, Erik, van der Lienden, Martijn J. C., Overkleeft, Herman S., van Kasteren, Sander I., Koster, Abraham J., Aerts, Johannes M. F. G.

“…β‐Glucocerebrosidase (GBA) is the enzyme that degrades glucosylceramide in lysosomes. Defects in GBA that result in overall loss of enzymatic activity give…”
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HEPES activates a MiT/TFE-dependent lysosomal-autophagic gene network in cultured cells: A call for caution by Tol, Marc J., van der Lienden, Martijn J.C., Gabriel, Tanit L., Hagen, Jacob J., Scheij, Saskia, Veenendaal, Tineke, Klumperman, Judith, Donker-Koopman, Wilma E., Verhoeven, Arthur J., Overkleeft, Hermen, Aerts, Johannes M., Argmann, Carmen A., van Eijk, Marco

“…In recent years, the lysosome has emerged as a highly dynamic, transcriptionally regulated organelle that is integral to nutrient-sensing and metabolic…”
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GCase and LIMP2 Abnormalities in the Liver of Niemann Pick Type C Mice by van der Lienden, Martijn J C, Aten, Jan, Marques, André R A, Waas, Ingeborg S E, Larsen, Per W B, Claessen, Nike, van der Wel, Nicole N, Ottenhoff, Roelof, van Eijk, Marco, Aerts, Johannes M F G

“…The lysosomal storage disease Niemann-Pick type C (NPC) is caused by impaired cholesterol efflux from lysosomes, which is accompanied by secondary lysosomal…”
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Bioorthogonal Correlative Light-Electron Microscopy of Mycobacterium tuberculosis in Macrophages Reveals the Effect of Antituberculosis Drugs on Subcellular Bacterial Distribution by Bakkum, Thomas, Heemskerk, Matthias T, Bos, Erik, Groenewold, Mirjam, Oikonomeas-Koppasis, Nikolaos, Walburg, Kimberley V, van Veen, Suzanne, van der Lienden, Martijn J. C, van Leeuwen, Tyrza, Haks, Marielle C, Ottenhoff, Tom H. M, Koster, Abraham J, van Kasteren, Sander I

“…Bioorthogonal correlative light-electron microscopy (B-CLEM) can give a detailed overview of multicomponent biological systems. It can provide information on…”
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Renal involvement in a patient with the chronic visceral subtype of acid sphingomyelinase deficiency resembles Fabry disease by Eskes, Eline C. B., Lienden, Martijn J. C., Roelofs, Joris J. T. H., Vogt, Liffert, Aerts, Johannes M. F. G., Aten, Jan, Hollak, Carla E. M.

“…Acid sphingomyelinase deficiency (ASMD) is a lysosomal storage disease (LSD) in which sphingomyelin accumulates due to deficient acid sphingomyelinase. In the…”
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