X
Search Filters
Format Format
Subjects Subjects
Subjects Subjects
X
Sort by Item Count (A-Z)
Filter by Count
index medicus (315) 315
cholesterol (289) 289
humans (256) 256
npc1 (231) 231
npc1 protein (218) 218
animals (204) 204
niemann-pick disease (176) 176
lipids (164) 164
mutation (145) 145
cholesterol - metabolism (136) 136
biochemistry & molecular biology (131) 131
mice (128) 128
proteins (115) 115
carrier proteins - genetics (112) 112
membrane glycoproteins - genetics (109) 109
cell biology (93) 93
niemann-pick disease, type c - genetics (89) 89
disease (87) 87
neurodegeneration (87) 87
protein (87) 87
male (83) 83
female (81) 81
trafficking (79) 79
genetics & heredity (72) 72
medicine (72) 72
hemic and lymphatic diseases (71) 71
niemann-pick disease, type c - metabolism (71) 71
sterol-sensing domain (71) 71
nutritional and metabolic diseases (68) 68
analysis (67) 67
carrier proteins - metabolism (67) 67
multidisciplinary sciences (65) 65
homeostasis (63) 63
transport (63) 63
membrane glycoproteins - metabolism (62) 62
congenital, hereditary, and neonatal diseases and abnormalities (60) 60
metabolism (59) 59
niemann-pick disease, type c - pathology (59) 59
proteins - genetics (59) 59
article (58) 58
genetic aspects (57) 57
lysosomes (57) 57
research article (56) 56
lysosomes - metabolism (55) 55
neurosciences (54) 54
purified npc1 protein (54) 54
research (54) 54
fibroblasts (53) 53
gene (53) 53
identification (53) 53
biology (52) 52
science (51) 51
binding (49) 49
proteins - metabolism (48) 48
disease type-c (47) 47
phenotype (46) 46
accumulation (43) 43
nervous system diseases (43) 43
npc2 (43) 43
physiological aspects (43) 43
brain (42) 42
disease models, animal (42) 42
genetics (42) 42
mice, knockout (42) 42
lipid metabolism (41) 41
cells (40) 40
glycoproteins - genetics (40) 40
mutations (40) 40
niemann-pick c1 (40) 40
adult (39) 39
cells, cultured (39) 39
genes (39) 39
medicine, research & experimental (38) 38
cell line (37) 37
mouse model (37) 37
neurodegenerative diseases (37) 37
neurons (37) 37
niemann-pick disease type c (37) 37
mice, inbred balb c (36) 36
plasma-membrane (36) 36
sterols (36) 36
child (35) 35
expression (35) 35
niemann-pick diseases - genetics (35) 35
biological transport (34) 34
biophysics (34) 34
cyclodextrin (34) 34
health aspects (34) 34
rodents (34) 34
mouse (33) 33
cholesterol homeostasis (32) 32
diagnosis (32) 32
niemann-pick diseases - metabolism (32) 32
niemann-pick type c disease (32) 32
child, preschool (31) 31
fibroblasts - metabolism (31) 31
liver (31) 31
niemann-pick disease, type c - drug therapy (31) 31
protein binding (31) 31
animal models (30) 30
more...
Language Language
Publication Date Publication Date
Click on a bar to filter by decade
Slide to change publication date range


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