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Nature Communications, ISSN 2041-1723, 12/2018, Volume 9, Issue 1, pp. 3671 - 13
Journal Article
Gut, ISSN 0017-5749, 06/2017, Volume 66, Issue 6, pp. 1060 - 1073
ObjectivePatients with Niemann–Pick disease type C1 (NPC1), a lysosomal lipid storage disorder that causes neurodegeneration and liver damage, can present with... 
IBD-GENETICS | IBD CLINICAL | IMMUNODEFICIENCY | IBD BASIC RESEARCH | Crohn'S DISEASE | MIGLUSTAT THERAPY | PATHOGENESIS | APOPTOSIS | DENDRITIC CELLS | MACROPHAGES | SUSCEPTIBILITY | ILEAL MUCOSA | PHAGOSOME PROTEOME | GASTROENTEROLOGY & HEPATOLOGY | BOWEL-DISEASE | INNATE IMMUNITY | Tumor Necrosis Factor-alpha - metabolism | Crohn Disease - genetics | X-Linked Inhibitor of Apoptosis Protein - deficiency | Humans | Child, Preschool | Lysosomes | Male | Nod2 Signaling Adaptor Protein - genetics | Leukocytes, Mononuclear | Pyridazines - pharmacology | Niemann-Pick Disease, Type C - physiopathology | Autophagy - drug effects | Crohn Disease - complications | Receptor-Interacting Protein Serine-Threonine Kinase 2 - metabolism | Young Adult | Gentamicins - pharmacology | Adult | Bacteria | Female | Genetic Diseases, X-Linked - genetics | Autophagy - genetics | Child | Receptor-Interacting Protein Serine-Threonine Kinase 2 - antagonists & inhibitors | Macrophages - physiology | Granuloma - genetics | Cells, Cultured | Chlorpromazine - pharmacology | Imidazoles - pharmacology | Acetylmuramyl-Alanyl-Isoglutamine - metabolism | X-Linked Inhibitor of Apoptosis Protein - genetics | Acetylmuramyl-Alanyl-Isoglutamine - pharmacology | Granuloma - pathology | Nod2 Signaling Adaptor Protein - metabolism | Crohn Disease - pathology | Dopamine Antagonists - pharmacology | Adolescent | X-Linked Inhibitor of Apoptosis Protein - metabolism | Macrophages - drug effects | Anti-Bacterial Agents - pharmacology | Protein Kinase Inhibitors - pharmacology | Mutation | Niemann-Pick Disease, Type C - complications | Niemann-Pick Disease, Type C - genetics | Autophagy (Cytology) | Inflammatory bowel diseases | Gastrointestinal diseases | Causes of | Colorectal diseases | Crohn's disease | Research | Niemann-Pick disease | Salmonella | Granuloma | Muramyl dipeptide | Liver | XIAP protein | Kinases | Macrophages | Autophagy | Defects | Genotype & phenotype | Cell activation | Intestine | Neurodegeneration | Npc1 protein | Age | NOD2 protein | Oligomerization | Dendritic cells | Cytokines | Blood & organ donations | Inflammation | Patients | Crohns disease | Studies | Inflammatory bowel disease | Monocytes | Proteomics | Colitis | Adapter proteins | Phagocytosis | Apoptosis | IBD - GENETICS | CROHN'S DISEASE | Inflammatory Bowel Disease | 1506
Journal Article
Cell, ISSN 0092-8674, 06/2016, Volume 165, Issue 6, pp. 1467 - 1478
Journal Article
Journal Article
Journal Article
FEBS Letters, ISSN 0014-5793, 2010, Volume 584, Issue 9, pp. 1700 - 1712
The constitutive degradation of membrane components takes place in the acidic compartments of a cell, the endosomes and lysosomes. Sites of lipid degradation... 
Saposin | GM2-activator | Endosome | Sphingolipid activator protein | Lysosome | Niemann–Pick disease, type C2-protein | lysosomal lipid-binding proteins | endosomal sorting complexes required for transport | glycosphingolipid | ORP | sterol carrier protein-2 | bis(monoacylglycero)phosphate | NBD | SCP-2 | 4-nitrobenzo-2-oxa-1,3-diazol | FRET | PtdIns | ganglioside-nomenclature according to Svennerholm, compare Fig. 1 for structures | GSL | sap | sn1, sn2 | BMP | ESCRT | oxysterol binding protein-related protein | sphingolipid activator protein (GM2-activator, sap-A–sap-D) | platelet activating factor | multivesicular bodies | fluorescence or Förster resonance energy transfer | NPC1 | PAF | phosphatidylinositol | NPC2 | stereospecific numbering of glycerolipids according to IUPAC | GM1, GM2, GM3 | LLBPs | Niemann–Pick disease, type C1-protein | saposin | MVBs | Niemann-Pick disease, type C2-protein | HUMAN SAPOSIN-C | INTRACELLULAR CHOLESTEROL TRAFFICKING | SPHINGOLIPID ACTIVATOR PROTEINS | ENDOCYTIC PATHWAY | BIOCHEMISTRY & MOLECULAR BIOLOGY | HUMAN GM2-ACTIVATOR PROTEIN | GAUCHER DISEASE | CELL BIOLOGY | NIEMANN-PICK-DISEASE | TRICYCLIC ANTIDEPRESSANT DESIPRAMINE | BIOPHYSICS | ACID BETA-GLUCOSIDASE | COENZYME Q10-BINDING/TRANSFER PROTEIN | G(M2) Activator Protein - metabolism | Signal Transduction | Humans | Glycoproteins - metabolism | Endocytosis - physiology | Saposins - metabolism | Animals | Carrier Proteins - metabolism | Lysosomes - metabolism | Models, Biological | Membrane Lipids - metabolism | Biological Transport - physiology | Membrane Lipids - immunology | Antigen Presentation - physiology | Lipid Metabolism - physiology
Journal Article