Experts and Doctors on lysosomes in Germany

Summary

Locale: Germany
Topic: lysosomes

Top Publications

  1. Trefzger J, Ronai A, Wassmer B, von Deimling O. Genetically defined lysosomal acetylesterase EC 3.1.1.6 in the cauda epididymidis of mouse, rat, and man. Histochemistry. 1992;98:373-9 pubmed
    ..1.1.6). Control experiments involving isoelectric focusing revealed that this acetylesterase was identical with the genetically defined homologues ES-17, ES-6, and ES-A4 in mouse, rat, and man, respectively. ..
  2. Saftig P, Hunziker E, Everts V, Jones S, Boyde A, Wehmeyer O, et al. Functions of cathepsin K in bone resorption. Lessons from cathepsin K deficient mice. Adv Exp Med Biol. 2000;477:293-303 pubmed
    ..Taken together the phenotype of cathepsin K knockout mice underlines the importance of this proteinase in bone remodelling. ..
  3. Lemansky P, Hasilik A. Chondroitin sulfate is involved in lysosomal transport of lysozyme in U937 cells. J Cell Sci. 2001;114:345-52 pubmed
    ..Our results suggest that the proteoglycan is involved in lysosomal targeting of lysozyme in U937 cells. ..
  4. Heit A, Schmitz F, O Keeffe M, Staib C, Busch D, Wagner H, et al. Protective CD8 T cell immunity triggered by CpG-protein conjugates competes with the efficacy of live vaccines. J Immunol. 2005;174:4373-80 pubmed
    ..These data underscore that the synergy imparted by CpG-OVA complex-mediated combined triggering of innate and specific immunity might be key to initiate CD8 T cell-based immunoprotection by synthetic vaccines based on subunit Ag. ..
  5. Lemansky P, Fester I, Smolenova E, Uhländer C, Hasilik A. The cation-independent mannose 6-phosphate receptor is involved in lysosomal delivery of serglycin. J Leukoc Biol. 2007;81:1149-58 pubmed
    ..We conclude that the CI-MPR participates in lysosomal and granular targeting of serglycin and basic proteins such as lysozyme associated with the proteoglycan in hematopoietic cells. ..
  6. Utermöhlen O, Herz J, Schramm M, Kronke M. Fusogenicity of membranes: the impact of acid sphingomyelinase on innate immune responses. Immunobiology. 2008;213:307-14 pubmed publisher
    ..In light of the biochemical and biophysical properties of ceramide, we provide a model suggesting that ASMase regulates select vesicular fusion processes by modifying the steric conformation of cellular membranes. ..
  7. Bauer C, Duewell P, Mayer C, Lehr H, Fitzgerald K, Dauer M, et al. Colitis induced in mice with dextran sulfate sodium (DSS) is mediated by the NLRP3 inflammasome. Gut. 2010;59:1192-9 pubmed publisher
    ..The NLRP3 inflammasome was identified as a critical mechanism of intestinal inflammation in the DSS colitis model. The NLRP3 inflammasome may serve as a potential target for the development of novel therapeutics for patients with IBD. ..
  8. Grabner A, Brast S, Sucic S, Bierer S, Hirsch B, Pavenstadt H, et al. LAPTM4A interacts with hOCT2 and regulates its endocytotic recruitment. Cell Mol Life Sci. 2011;68:4079-90 pubmed publisher
    ..In this work, LAPTM4A has been identified as interaction partner of hOCT2. LAPTM4A regulates the function of hOCT2 by influencing its trafficking to/from the cell membrane and processing it via the intracellular sorting machinery. ..
  9. Gonzalez P, Mader I, Tchoghandjian A, Enzenmüller S, Cristofanon S, Basit F, et al. Impairment of lysosomal integrity by B10, a glycosylated derivative of betulinic acid, leads to lysosomal cell death and converts autophagy into a detrimental process. Cell Death Differ. 2012;19:1337-46 pubmed publisher
    ..These findings have important implications for the therapeutic exploitation of BA derivatives, particularly in apoptosis-resistant cancers. ..

More Information

Publications124 found, 100 shown here

  1. Poeter M, Radke S, Koese M, Hessner F, Hegemann A, Musiol A, et al. Disruption of the annexin A1/S100A11 complex increases the migration and clonogenic growth by dysregulating epithelial growth factor (EGF) signaling. Biochim Biophys Acta. 2013;1833:1700-11 pubmed publisher
    ..This article is part of a Special Issue entitled: 12th European Symposium on Calcium. ..
  2. Schulze H, Sandhoff K. Sphingolipids and lysosomal pathologies. Biochim Biophys Acta. 2014;1841:799-810 pubmed publisher
    ..IMs are rich in anionic bis(monoacylglycero)phosphate (BMP). This article is part of a Special Issue entitled New Frontiers in Sphingolipid Biology. ..
  3. Muntoni S, Wiebusch H, Funke H, Ros E, Seedorf U, Assmann G. Homozygosity for a splice junction mutation in exon 8 of the gene encoding lysosomal acid lipase in a Spanish kindred with cholesterol ester storage disease (CESD). Hum Genet. 1995;95:491-4 pubmed
    ..Nevertheless, our findings provide convincing evidence that homozygosity for the E8SJM-allele causes cholesterol ester storage disease to at least the same extent as compound heterozygosity consisting of this allele and a null allele. ..
  4. Kopitz J, von Reitzenstein C, Muhl C, Cantz M. Role of plasma membrane ganglioside sialidase of human neuroblastoma cells in growth control and differentiation. Biochem Biophys Res Commun. 1994;199:1188-93 pubmed
    ..The results suggest an important role of the ganglioside sialidase of the plasma membrane in the processes of proliferation control and differentiation in this neuronal cell system. ..
  5. Klima H, Ullrich K, Aslanidis C, Fehringer P, Lackner K, Schmitz G. A splice junction mutation causes deletion of a 72-base exon from the mRNA for lysosomal acid lipase in a patient with cholesteryl ester storage disease. J Clin Invest. 1993;92:2713-8 pubmed
    ..In summary, the data presented provide evidence that deletion of the codons for amino acids 254-277 in the LAL mRNA in combination with a null allele cause the clinical expression of CESD in our patient. ..
  6. Bispinck F, Fischer J, Lullmann Rauch R, Ziegenhagen M. Time course of the tilorone-induced lysosomal accumulation of sulphated glycosaminoglycans in cultured fibroblasts. Exp Toxicol Pathol. 1998;50:411-5 pubmed
    ..This is thought to be due to the fact that the temporal development of storage of undigested GAGs depends on the natural delivery of GAGs towards the lysosomal apparatus. ..
  7. Dittmer F, Ulbrich E, Hafner A, Schmahl W, Meister T, Pohlmann R, et al. Alternative mechanisms for trafficking of lysosomal enzymes in mannose 6-phosphate receptor-deficient mice are cell type-specific. J Cell Sci. 1999;112 ( Pt 10):1591-7 pubmed
  8. Thumann G, Bartz Schmidt K, Kociok N, Heimann K, Schraemeyer U. Ultimate fate of rod outer segments in the retinal pigment epithelium. Pigment Cell Res. 1999;12:311-5 pubmed
    ..These results indicate that, in RPE, in vivo degradation of ROS is associated with melanosomes. ..
  9. Welss T, Sun J, Irving J, Blum R, Smith A, Whisstock J, et al. Hurpin is a selective inhibitor of lysosomal cathepsin L and protects keratinocytes from ultraviolet-induced apoptosis. Biochemistry. 2003;42:7381-9 pubmed
    ..Given that lysosomal disruption, release of catL, and catL-mediated caspase activation are known to occur in response to cellular stress, we propose that a physiological role of hurpin is to protect epithelial cells from ectopic catL. ..
  10. Fortunato F, Berger I, Gross M, Rieger P, Buechler M, Werner J. Immune-compromised state in the rat pancreas after chronic alcohol exposure: the role of peroxisome proliferator-activated receptor gamma. J Pathol. 2007;213:441-52 pubmed
    ..Intracellular mitochondrial and lysosomal damage after chronic alcohol exposure induces premature activation of digestive enzymes and establishment of peri-cellular fibrosis in the absence of inflammation. ..
  11. Fortunato F, Kroemer G. Impaired autophagosome-lysosome fusion in the pathogenesis of pancreatitis. Autophagy. 2009;5:850-3 pubmed
    ..Hence, we postulate that the depletion of lysosomal proteins may play a critical role in the pathogenesis of acute pancreatitis. ..
  12. Zhao Y, Könen Waisman S, Taylor G, Martens S, Howard J. Localisation and mislocalisation of the interferon-inducible immunity-related GTPase, Irgm1 (LRG-47) in mouse cells. PLoS ONE. 2010;5:e8648 pubmed publisher
    ..In future analyses it should be borne in mind that tagging of Irgm1 leads to loss of Golgi localisation and enhanced localisation on endolysosomal membranes, probably as a result of constitutive activation. ..
  13. Demirel O, Bangert I, Tampe R, Abele R. Tuning the cellular trafficking of the lysosomal peptide transporter TAPL by its N-terminal domain. Traffic. 2010;11:383-93 pubmed publisher
    ..Therefore, TMD0 represents a unique domain, which folds independently and encodes the information for lysosomal targeting. These outcomes are discussed in respect of trafficking, folding and function of TAPL...
  14. Saftig P, Hetman M, Schmahl W, Weber K, Heine L, Mossmann H, et al. Mice deficient for the lysosomal proteinase cathepsin D exhibit progressive atrophy of the intestinal mucosa and profound destruction of lymphoid cells. EMBO J. 1995;14:3599-608 pubmed
  15. Muller R, Jacobs C, Kayser O. Nanosuspensions as particulate drug formulations in therapy. Rationale for development and what we can expect for the future. Adv Drug Deliv Rev. 2001;47:3-19 pubmed
    ..The possibilities of large scale production -- the prerequisite for the introduction of a delivery system to the market -- are also discussed. ..
  16. Heine D, Muller R, Brüsselbach S. Cell surface display of a lysosomal enzyme for extracellular gene-directed enzyme prodrug therapy. Gene Ther. 2001;8:1005-10 pubmed
    ..Using a doxorubicin prodrug, we demonstrate that this GDEPT system produces a strong bystander effect and has potent antitumor activity in vivo. ..
  17. Classen C, Falk C, Friesen C, Fulda S, Herr I, Debatin K. Natural killer resistance of a drug-resistant leukemia cell line, mediated by up-regulation of HLA class I expression. Haematologica. 2003;88:509-21 pubmed
    ..This finding may have a clinical impact since it may be considered as a possible reason for resistance to a graft-versus-leukemia approach in allogeneic bone marrow transplantation ..
  18. Bräuer A, Nitsch R, Savaskan N. Identification of macrophage/microglia activation factor (MAF) associated with late endosomes/lysosomes in microglial cells. FEBS Lett. 2004;563:41-8 pubmed
    ..In conclusion, these results imply that MAF is involved in the dynamics of lysosomal membranes associated with microglial activation following brain lesion. ..
  19. Schwartz V, Friedrich K, Polleichtner G, Gründer S. Acid-sensing ion channel (ASIC) 4 predominantly localizes to an early endosome-related organelle upon heterologous expression. Sci Rep. 2015;5:18242 pubmed publisher
    ..Collectively, our results show that heterologously expressed ASIC4 predominantly resides in an intracellular endosomal compartment. ..
  20. Schaefer L, Han X, Gretz N, Schaefer R. Alterations of cathepsins B, H and L in proximal tubules from polycystic kidneys of the Han:SPRD rat. Kidney Int. 1996;50:424-31 pubmed
  21. Thevenod F, Friedmann J. Cadmium-mediated oxidative stress in kidney proximal tubule cells induces degradation of Na+/K(+)-ATPase through proteasomal and endo-/lysosomal proteolytic pathways. FASEB J. 1999;13:1751-61 pubmed
    ..Degradation of oxidatively damaged Na+/K(+)-ATPase may contribute to the 'Fanconi syndrome'-like Na(+)-dependent transport defects associated with Cd-nephrotoxicity. ..
  22. Lautwein A, Kraus M, Reich M, Burster T, Brandenburg J, Overkleeft H, et al. Human B lymphoblastoid cells contain distinct patterns of cathepsin activity in endocytic compartments and regulate MHC class II transport in a cathepsin S-independent manner. J Leukoc Biol. 2004;75:844-55 pubmed
    ..Thus, BLC contain distinct activity patterns of proteases in endocytic compartments and regulate the intracellular transport and surface-delivery of class II in a CatS-independent manner. ..
  23. Schröder B, Hasilik A. A protocol for combined delipidation and subfractionation of membrane proteins using organic solvents. Anal Biochem. 2006;357:144-6 pubmed
  24. Steenhuis P, Froemming J, Reinheckel T, Storch S. Proteolytic cleavage of the disease-related lysosomal membrane glycoprotein CLN7. Biochim Biophys Acta. 2012;1822:1617-28 pubmed publisher
    ..Our findings suggest that CLN7 is inactivated by proteolytic cleavage and that enhanced CLN7 proteolysis caused by missense mutations in selected luminal loops is associated with disease. ..
  25. Baumgrass R, Williamson M, Price P. Identification of peptide fragments generated by digestion of bovine and human osteocalcin with the lysosomal proteinases cathepsin B, D, L, H, and S. J Bone Miner Res. 1997;12:447-55 pubmed
    ..These results demonstrate the utility of peptides generated by cathepsin digestion in the mapping of the antigenic epitopes recognized by a given BGP immunoassay. ..
  26. Santama N, Krijnse Locker J, Griffiths G, Noda Y, Hirokawa N, Dotti C. KIF2beta, a new kinesin superfamily protein in non-neuronal cells, is associated with lysosomes and may be implicated in their centrifugal translocation. EMBO J. 1998;17:5855-67 pubmed
    ..These results implicate KIF2beta as a motor responsible for the peripheral translocation of lysosomes. ..
  27. Burster T, Beck A, Poeschel S, Øren A, Baechle D, Reich M, et al. Interferon-gamma regulates cathepsin G activity in microglia-derived lysosomes and controls the proteolytic processing of myelin basic protein in vitro. Immunology. 2007;121:82-93 pubmed
    ..Thus, the cytokine environment modulates lysosomal proteases in microglia by a selective down-regulation of CatG, leading to decreased MBP-processing by microglia-derived lysosomal proteases in vitro. ..
  28. Schnöder L, Hao W, Qin Y, Liu S, Tomic I, Liu X, et al. Deficiency of Neuronal p38α MAPK Attenuates Amyloid Pathology in Alzheimer Disease Mouse and Cell Models through Facilitating Lysosomal Degradation of BACE1. J Biol Chem. 2016;291:2067-79 pubmed publisher
    ..Thus, our study demonstrates that p38α MAPK plays a critical role in the regulation of BACE1 degradation and Aβ generation in AD pathogenesis. ..
  29. Weide T, Huber T. Implications of autophagy for glomerular aging and disease. Cell Tissue Res. 2011;343:467-73 pubmed publisher
    ..This review focuses on recent findings that identify autophagy as a critical homeostatic and quality control mechanism maintaining glomerular homeostasis. ..
  30. Gil P, Nazarenus M, Ashraf S, Parak W. pH-sensitive capsules as intracellular optical reporters for monitoring lysosomal pH changes upon stimulation. Small. 2012;8:943-8 pubmed publisher
    ..With help of this sensor concept it is demonstrated that the different agents used (Monensin, Chloroquine, Bafilomycin A1, Amiloride) possessed different kinetics and mechanisms of action in affecting the intracellular pH values. ..
  31. Koster A, Von Figura K, Pohlmann R. Mistargeting of lysosomal enzymes in M(r) 46,000 mannose 6-phosphate receptor-deficient mice is compensated by carbohydrate-specific endocytotic receptors. Eur J Biochem. 1994;224:685-9 pubmed
    ..Moreover, evidence was obtained that, also in control mice, the steady-state level of some lysosomal enzyme is controlled by these receptors. ..
  32. Schröder B, Wrocklage C, Pan C, Jäger R, Kösters B, Schafer H, et al. Integral and associated lysosomal membrane proteins. Traffic. 2007;8:1676-86 pubmed
    ..Finally, our results identified a particular set of proteins with known functions in signaling and targeting to be at least partially associated with lysosomes. ..
  33. Pohl C, Jentsch S. Midbody ring disposal by autophagy is a post-abscission event of cytokinesis. Nat Cell Biol. 2009;11:65-70 pubmed publisher
    ..Thus our findings suggest that autophagy has a broader role than previously assumed, and that cell renovation by clearing from superfluous large macromolecular assemblies, such as MRs, is an important autophagic function. ..
  34. Schwarz G, Boehncke W, Braun M, Schröter C, Burster T, Flad T, et al. Cathepsin S activity is detectable in human keratinocytes and is selectively upregulated upon stimulation with interferon-gamma. J Invest Dermatol. 2002;119:44-9 pubmed
    ..Our observations support the concept of keratinocytes functioning as nonprofessional antigen-presenting cells in states of inflammation. ..
  35. Böttcher R, Stremmel C, Meves A, Meyer H, Widmaier M, Tseng H, et al. Sorting nexin 17 prevents lysosomal degradation of ?1 integrins by binding to the ?1-integrin tail. Nat Cell Biol. 2012;14:584-92 pubmed publisher
    ..Our results identify SNX17 as a ?1-integrin-tail-binding protein that interacts with the free Kindlin-binding site in endosomes to stabilize ?1 integrins, resulting in their recycling to the cell surface where they can be reused. ..
  36. Kurschus F, Bruno R, Fellows E, Falk C, Jenne D. Membrane receptors are not required to deliver granzyme B during killer cell attack. Blood. 2005;105:2049-58 pubmed
    ..We conclude that membrane receptors for GzmB on target cells are not crucial for killer cell-mediated apoptosis. ..
  37. Ramirez A, Heimbach A, Gründemann J, Stiller B, Hampshire D, Cid L, et al. Hereditary parkinsonism with dementia is caused by mutations in ATP13A2, encoding a lysosomal type 5 P-type ATPase. Nat Genet. 2006;38:1184-91 pubmed
    ..Our findings link a class of proteins with unknown function and substrate specificity to the protein networks implicated in neurodegeneration and parkinsonism. ..
  38. Karbach S, Simon A, Slenzka A, Jaenecke I, Habermeier A, Martine U, et al. Relative contribution of different l-arginine sources to the substrate supply of endothelial nitric oxide synthase. J Mol Cell Cardiol. 2011;51:855-61 pubmed publisher
    ..Our study thus demonstrates that l-citrulline and l-arginine-containing peptides derived from either intracellular protein breakdown or from the extracellular space seem to be good substrate sources for eNOS. ..
  39. Lullmann Rauch R, Pods R, von Witzendorff B. The antimalarials quinacrine and chloroquine induce weak lysosomal storage of sulphated glycosaminoglycans in cell culture and in vivo. Toxicology. 1996;110:27-37 pubmed
    ..This suggests that the asymmetric structure of the quinacrine molecule reduces the potency as compared to the symmetrically substituted bisbasic compounds with planary tricyclic ring systems such as tilorone and congeners. ..
  40. Klein D, Lichtmannegger J, Heinzmann U, Summer K. Dissolution of copper-rich granules in hepatic lysosomes by D-penicillamine prevents the development of fulminant hepatitis in Long-Evans cinnamon rats. J Hepatol. 2000;32:193-201 pubmed
    ..However, the mobilization of copper by D-penicillamine seems to be limited due to the binding of the metal to metallothionein in liver cytosol. This copper, even at relatively high concentrations, apparently may be well tolerated. ..
  41. Bispinck F, Fischer J, Lullmann Rauch R, von Witzendorff B. Lysosomal glycosaminoglycan storage as induced by dicationic amphiphilic drugs: investigation into the mechanisms underlying the slow reversibility. Toxicology. 1998;128:91-100 pubmed
    ..The present results suggest that the persistence of the DS storage is due to the formation of long-lived, non-degradable DS-drug complexes within the lysosomes. ..
  42. Mayer K, Vreemann A, Qu H, Brix K. Release of endo-lysosomal cathepsins B, D, and L from IEC6 cells in a cell culture model mimicking intestinal manipulation. Biol Chem. 2009;390:471-80 pubmed publisher
    ..We conclude that our apparatus for mechanical manipulation can be used to approach surgical trauma, thereby focusing on epithelial cells of the intestine mucosa. ..
  43. Clement A, Gamerdinger M, Tamboli I, Lutjohann D, Walter J, Greeve I, et al. Adaptation of neuronal cells to chronic oxidative stress is associated with altered cholesterol and sphingolipid homeostasis and lysosomal function. J Neurochem. 2009;111:669-82 pubmed publisher
    ..These findings suggest an important role of the cell type-specific lipid profile for differential vulnerabilities of different brain areas toward chronic oxidative stress. ..
  44. König J, Besoke F, Stuetz W, Malarski A, Jahreis G, Grune T, et al. Quantification of age-related changes of ?-tocopherol in lysosomal membranes in murine tissues and human fibroblasts. Biofactors. 2016;42:307-15 pubmed publisher
    ..Furthermore saturation and intracellular distribution of ?-Toc seem to be strongly dependent on the availability of this vitamin as well as on the presence of the lysosomal protein NPC1. © 2016 BioFactors, 42(3):307-315, 2016. ..
  45. Handrock K, Laschke A, Lullmann Rauch R, Vogt R, Ziegenhagen M. Lysosomal storage of sulfated glycosaminoglycans in cultured fibroblasts exposed to immunostimulatory acridine derivatives. Toxicol Appl Pharmacol. 1992;114:204-14 pubmed
  46. Tawo R, Pokrzywa W, Kevei E, Akyuz M, Balaji V, Adrian S, et al. The Ubiquitin Ligase CHIP Integrates Proteostasis and Aging by Regulation of Insulin Receptor Turnover. Cell. 2017;169:470-482.e13 pubmed publisher
    ..Our study indicates a competitive relationship between proteostasis and longevity regulation through CHIP-assisted proteolysis, providing a mechanistic concept for understanding the impact of proteome imbalance on aging. ..
  47. Di Piazza M, Mader C, Geletneky K, Herrero Y Calle M, Weber E, Schlehofer J, et al. Cytosolic activation of cathepsins mediates parvovirus H-1-induced killing of cisplatin and TRAIL-resistant glioma cells. J Virol. 2007;81:4186-98 pubmed
  48. Chapuy B, Koch R, Radunski U, Corsham S, Cheong N, Inagaki N, et al. Intracellular ABC transporter A3 confers multidrug resistance in leukemia cells by lysosomal drug sequestration. Leukemia. 2008;22:1576-86 pubmed publisher
    ..In conclusion, we identified subcellular drug sequestration mediated by the genuinely intracellular ABCA3 as being a clinically relevant mechanism of intrinsic MDR...
  49. Grune T, Merker K, Jung T, Sitte N, Davies K. Protein oxidation and degradation during postmitotic senescence. Free Radic Biol Med. 2005;39:1208-15 pubmed
    ..This decline in proteolytic capacity was accompanied by an increased accumulation of oxidized proteins. ..
  50. Schröder B, Wrocklage C, Hasilik A, Saftig P. Molecular characterisation of 'transmembrane protein 192' (TMEM192), a novel protein of the lysosomal membrane. Biol Chem. 2010;391:695-704 pubmed publisher
    ..TMEM192 was found to be strongly expressed in human kidney, liver, lung and pancreas tissue. The widespread tissue distribution could suggest an important role of TMEM192 for lysosomal function. ..
  51. Bangert I, Tumulka F, Abele R. The lysosomal polypeptide transporter TAPL: more than a housekeeping factor?. Biol Chem. 2011;392:61-6 pubmed publisher
    ..Additionally, we will discuss its proposed physiological functions such as housekeeping together with a specialized factor for metabolite storage as well as for the adaptive immunity...
  52. Tamhane T, Lllukkumbura R, Lu S, Maelandsmo G, Haugen M, Brix K. Nuclear cathepsin L activity is required for cell cycle progression of colorectal carcinoma cells. Biochimie. 2016;122:208-18 pubmed publisher
    ..Furthermore, we hypothesize that nuclear cathepsin L accelerates cell cycle progression of HCT116 cells thereby supporting the notion that cysteine cathepsins may play significant roles in carcinogenesis due to deregulated trafficking. ..
  53. Sender V, Moulakakis C, Stamme C. Pulmonary surfactant protein A enhances endolysosomal trafficking in alveolar macrophages through regulation of Rab7. J Immunol. 2011;186:2397-411 pubmed publisher
    ..The data demonstrate a novel role for SP-A in modulating endolysosomal trafficking via Rab7 in primary AM and define biochemical pathways involved. ..
  54. Jenne N, Rauchenberger R, Hacker U, Kast T, Maniak M. Targeted gene disruption reveals a role for vacuolin B in the late endocytic pathway and exocytosis. J Cell Sci. 1998;111 ( Pt 1):61-70 pubmed
    ..Furthermore, in the mutants post-lysosomal vacuoles are dramatically increased in size and accumulate endocytic marker, suggesting a role for vacuolin B in targeting the vacuole for exocytosis. ..
  55. Dittmer F, Hafner A, Ulbrich E, Moritz J, Schmidt P, Schmahl W, et al. I-cell disease-like phenotype in mice deficient in mannose 6-phosphate receptors. Transgenic Res. 1998;7:473-83 pubmed
    ..Inspite of the partial reactivation the phenotype of these mice was similar to that of triple deficient mice. ..
  56. Ludwig A, Ehlert J, Flad H, Brandt E. Identification of distinct surface-expressed and intracellular CXC-chemokine receptor 2 glycoforms in neutrophils: N-glycosylation is essential for maintenance of receptor surface expression. J Immunol. 2000;165:1044-52 pubmed
    ..Thus, although not directly involved in signaling, glycosylation appears to be required to maintain neutrophil responsiveness to CXC-chemokines during inflammation. ..
  57. Schmitz G, Kaminski W. ABCA2: a candidate regulator of neural transmembrane lipid transport. Cell Mol Life Sci. 2002;59:1285-95 pubmed
    ..The unique expression profile together with available structural data suggest roles for this largest known ABC protein in neural transmembrane lipid export...
  58. Biederbick A, Rösser R, Storre J, Elsässer H. The VSFASSQQ motif confers calcium sensitivity to the intracellular apyrase LALP70. BMC Biochem. 2004;5:8 pubmed
    ..Whether this is due to direct binding of calcium to this motif or to a conformational change of the enzyme, remains to be elucidated. ..
  59. Willenborg M, Schmidt C, Braun P, Landgrebe J, von Figura K, Saftig P, et al. Mannose 6-phosphate receptors, Niemann-Pick C2 protein, and lysosomal cholesterol accumulation. J Lipid Res. 2005;46:2559-69 pubmed
    ..In addition, we observed an upregulation of NPC1 protein and mRNA in the MPR-double-deficient cells. Taken together, our results suggest that the lysosomal targeting of NPC2 is strictly dependent on MPRs in fibroblasts. ..
  60. Nunes F, Wolf M, Hartmann J, Paul R. The ABC transporter PGP-2 from Caenorhabditis elegans is expressed in the sensory neuron pair AWA and contributes to lysosome formation and lipid storage within the intestine. Biochem Biophys Res Commun. 2005;338:862-71 pubmed
    ..Our results imply that PGP-2 is involved in a signalling process that connects sensory inputs to intestinal functions, possibly by influencing acidification of intestinal lysosomes, which in turn may affect pinocytosis and lipid storage...
  61. Poet M, Kornak U, Schweizer M, Zdebik A, Scheel O, Hoelter S, et al. Lysosomal storage disease upon disruption of the neuronal chloride transport protein ClC-6. Proc Natl Acad Sci U S A. 2006;103:13854-9 pubmed
    ..CLCN6 is a candidate gene for mild forms of human NCL. Analysis of 75 NCL patients identified ClC-6 amino acid exchanges in two patients but failed to prove a causative role of CLCN6 in that disease. ..
  62. Deuschl F, Kollmann K, von Figura K, Lübke T. Molecular characterization of the hypothetical 66.3-kDa protein in mouse: lysosomal targeting, glycosylation, processing and tissue distribution. FEBS Lett. 2006;580:5747-52 pubmed
    ..Interestingly, in mouse the endogenous 66.3-kDa protein is processed in a highly tissue-dependent manner to mature forms. ..
  63. Schafer P, Cymerman I, Bujnicki J, Meiss G. Human lysosomal DNase IIalpha contains two requisite PLD-signature (HxK) motifs: evidence for a pseudodimeric structure of the active enzyme species. Protein Sci. 2007;16:82-91 pubmed
    ..Based on these data, we present an experimentally validated structural model of DNase IIalpha. ..
  64. Jalil Y, Ritz V, Jakimenko A, Schmitz Salue C, Siebert H, Awuah D, et al. Vesicular localization of the rat ATP-binding cassette half-transporter rAbcb6. Am J Physiol Cell Physiol. 2008;294:C579-90 pubmed
    ..In summary, these results demonstrate that rAbcb6 is a glycosylated protein targeted to intracellular vesicular membranes and suggest involvement of rAbcb6 in transition metal homeostasis...
  65. Hoermannsperger G, Hörmannsperger G, Clavel T, Hoffmann M, Reiff C, Kelly D, et al. Post-translational inhibition of IP-10 secretion in IEC by probiotic bacteria: impact on chronic inflammation. PLoS ONE. 2009;4:e4365 pubmed publisher
    ..Furthermore, we revealed post-translational degradation of IP-10 protein in IEC to be the molecular mechanism underlying the anti-inflammatory effect. ..
  66. Schieder M, Rötzer K, Brüggemann A, Biel M, Wahl Schott C. Characterization of two-pore channel 2 (TPCN2)-mediated Ca2+ currents in isolated lysosomes. J Biol Chem. 2010;285:21219-22 pubmed publisher
    ..Our glass chip-based method will provide electrophysiological access not only to lysosomal TPCN channels but also to a broad range of other intracellular ion channels. ..
  67. Bauer S. Toll-like receptor 9 processing: the key event in Toll-like receptor 9 activation?. Immunol Lett. 2013;149:85-7 pubmed publisher
    ..However, mutants of TLR9 that do not affect proteolytic cleavage lost their functionality suggesting a more complex scenario of TLR9 activation. ..
  68. Laetz E, Wägele H. Chloroplast digestion and the development of functional kleptoplasty in juvenile Elysia timida (Risso, 1818) as compared to short-term and non-chloroplast-retaining sacoglossan slugs. PLoS ONE. 2017;12:e0182910 pubmed publisher
  69. Aumuller G, Renneberg H, Hasilik A. Distribution and subcellular localization of a lysosome-associated protein in human genital organs. Cell Tissue Res. 1997;287:335-42 pubmed
    ..The findings indicate that in the human prostate most of the membrane-bound lamp 2 is released from the secretory cells, presumably in an apocrine fashion. ..
  70. Stypmann J, Gläser K, Roth W, Tobin D, Petermann I, Matthias R, et al. Dilated cardiomyopathy in mice deficient for the lysosomal cysteine peptidase cathepsin L. Proc Natl Acad Sci U S A. 2002;99:6234-9 pubmed
    ..Furthermore, abnormal heart rhythms, like supraventricular tachycardia, ventricular extrasystoles, and first-degree atrioventricular block, were detected in the CTSL-deficient mice. ..
  71. Uhl J, Penzel R, Sergi C, Kopitz J, Otto H, Cantz M. Identification of a CTL4/Neu1 fusion transcript in a sialidosis patient. FEBS Lett. 2002;521:19-23 pubmed
    ..In one patient we found the resulting CTL4/Neu1 fusion transcript, in the other we detected an alternatively spliced CTL4 transcript (retention of intron 9). ..
  72. Linke M, Herzog V, Brix K. Trafficking of lysosomal cathepsin B-green fluorescent protein to the surface of thyroid epithelial cells involves the endosomal/lysosomal compartment. J Cell Sci. 2002;115:4877-89 pubmed
  73. Eskelinen E, Tanaka Y, Saftig P. At the acidic edge: emerging functions for lysosomal membrane proteins. Trends Cell Biol. 2003;13:137-45 pubmed
    ..The pivotal function of lysosomal membrane proteins is also highlighted by the recent identification of disease-causing mutations in cystine and sialic acid transporter proteins, leading to nephropathic cystinosis and Salla disease...
  74. Scharschmidt E, Wegener E, Heissmeyer V, Rao A, Krappmann D. Degradation of Bcl10 induced by T-cell activation negatively regulates NF-kappa B signaling. Mol Cell Biol. 2004;24:3860-73 pubmed
    ..Together, these results suggest a new mechanism of negative signaling in which TCR/PKC signaling initially activates Bcl10 but later promotes its degradation. ..
  75. Storch S, Pohl S, Braulke T. A dileucine motif and a cluster of acidic amino acids in the second cytoplasmic domain of the batten disease-related CLN3 protein are required for efficient lysosomal targeting. J Biol Chem. 2004;279:53625-34 pubmed
  76. Crosetto N, Tikkanen R, Dikic I. Oncogenic breakdowns in endocytic adaptor proteins. FEBS Lett. 2005;579:3231-8 pubmed
    ..We discuss how breakdowns in the function of endocytic adaptors might facilitate impairment of tissue homeostasis and consequent tumor development. ..
  77. Körner U, Fuss V, Steigerwald J, Moll H. Biogenesis of Leishmania major-harboring vacuoles in murine dendritic cells. Infect Immun. 2006;74:1305-12 pubmed
    ..The differences in the fusion competences of immature and mature DC may be relevant for their distinct functional activities in the uptake, transport, and presentation of parasite antigens. ..
  78. Petermann I, Mayer C, Stypmann J, Biniossek M, Tobin D, Engelen M, et al. Lysosomal, cytoskeletal, and metabolic alterations in cardiomyopathy of cathepsin L knockout mice. FASEB J. 2006;20:1266-8 pubmed
    ..Lysosomal impairment in ctsl-/- hearts results in metabolic and sarcomeric alterations that promote DCM development. ..
  79. Stahl S, Reinders Y, Asan E, Mothes W, Conzelmann E, Sickmann A, et al. Proteomic analysis of cathepsin B- and L-deficient mouse brain lysosomes. Biochim Biophys Acta. 2007;1774:1237-46 pubmed
  80. Riedel A, Nimmerjahn F, Burdach S, Behrends U, Bornkamm G, Mautner J. Endogenous presentation of a nuclear antigen on MHC class II by autophagy in the absence of CRM1-mediated nuclear export. Eur J Immunol. 2008;38:2090-5 pubmed publisher
    ..Thus, this endogenous presentation pathway broadens the spectrum of intracellular antigens surveyed by CD4(+) T cells by efficiently sampling cytoplasmic as well as nuclear antigens. ..
  81. Rambold A, Miesbauer M, Olschewski D, Seidel R, Riemer C, Smale L, et al. Green tea extracts interfere with the stress-protective activity of PrP and the formation of PrP. J Neurochem. 2008;107:218-29 pubmed publisher
    ..Our study emphasizes the important role of PrP(C) to protect cells from stress and indicate efficient intracellular pathways to degrade non-native conformations of PrP(C). ..
  82. Kettern N, Dreiseidler M, Tawo R, Hohfeld J. Chaperone-assisted degradation: multiple paths to destruction. Biol Chem. 2010;391:481-9 pubmed publisher
    ..Here, we discuss recent insights into molecular mechanisms underlying chaperone-assisted degradation in mammalian cells and highlight its biomedical relevance. ..
  83. Bögershausen N, Shahrzad N, Chong J, von Kleist Retzow J, Stanga D, Li Y, et al. Recessive TRAPPC11 mutations cause a disease spectrum of limb girdle muscular dystrophy and myopathy with movement disorder and intellectual disability. Am J Hum Genet. 2013;93:181-90 pubmed publisher
  84. Hille Rehfeld A. Mannose 6-phosphate receptors in sorting and transport of lysosomal enzymes. Biochim Biophys Acta. 1995;1241:177-94 pubmed
    ..To understand the physiological relevance of these observations will be a challenge for future research. ..
  85. Bernardo K, Hurwitz R, Zenk T, Desnick R, Ferlinz K, Schuchman E, et al. Purification, characterization, and biosynthesis of human acid ceramidase. J Biol Chem. 1995;270:11098-102 pubmed
    ..A minor portion of newly synthesized acid ceramidase was secreted into the medium as a monomeric 47-kDa protein, indicating that generation of the mature heterodimeric enzyme occurred in endosomal and/or lysosomal compartments. ..
  86. Koster A, Saftig P, Matzner U, Von Figura K, Peters C, Pohlmann R. Targeted disruption of the M(r) 46,000 mannose 6-phosphate receptor gene in mice results in misrouting of lysosomal proteins. EMBO J. 1993;12:5219-23 pubmed
    ..The phenotype of MPR46 -/- mice was normal, indicating mechanisms that compensate the MPR46 deficiency in vivo. ..
  87. Moolenaar C, Ouwendijk J, Wittpoth M, Wisselaar H, Hauri H, Ginsel L, et al. A mutation in a highly conserved region in brush-border sucrase-isomaltase and lysosomal alpha-glucosidase results in Golgi retention. J Cell Sci. 1997;110 ( Pt 5):557-67 pubmed
  88. Stoffel B, Bauer P, Nix M, Deres K, Stoffel W. Ceramide-independent CD28 and TCR signaling but reduced IL-2 secretion in T cells of acid sphingomyelinase-deficient mice. Eur J Immunol. 1998;28:874-80 pubmed
    ..We conclude that ceramide generated by aSMase is not involved in CD28 signal transduction, but rather a perturbation of the secretory system is responsible for the impaired proliferation of aSMase-deficient splenocytes. ..
  89. Roosen A, Schober A, Strelau J, Bottner M, Faulhaber J, Bendner G, et al. Lack of neurotrophin-4 causes selective structural and chemical deficits in sympathetic ganglia and their preganglionic innervation. J Neurosci. 2001;21:3073-84 pubmed
    ..Impairment of synaptic connectivity may consequently reduce impulse flow, causing a reduction in transmitter synthesis in postganglionic neurons. ..
  90. Schott I, Hartmann D, Gieselmann V, Lullmann Rauch R. Sulfatide storage in visceral organs of arylsulfatase A-deficient mice. Virchows Arch. 2001;439:90-6 pubmed
    ..In addition to being an animal model of the human disease, the ASA-deficient mouse may be useful for investigating the cell biology of sulfolipids in visceral organs...
  91. Rothe G, Klouche M. Phagocyte function. Methods Cell Biol. 2004;75:679-708 pubmed
  92. Lange P, Wartosch L, Jentsch T, Fuhrmann J. ClC-7 requires Ostm1 as a beta-subunit to support bone resorption and lysosomal function. Nature. 2006;440:220-3 pubmed
    ..3). The finding that grey-lethal mice, just like ClC-7-deficient mice, show lysosomal storage and neurodegeneration in addition to osteopetrosis implies a more general importance for ClC-7-Ostm1 complexes. ..