Articles

Search Articles

Advanced search
Help

Catalogue

Articles

Databases

X
Search Filters
Format Format
Format Format
X
Sort by Item Count (A-Z)
Filter by Count
Journal Article (579) 579
Newsletter (190) 190
Publication (46) 46
Book Chapter (22) 22
Book Review (12) 12
Newspaper Article (12) 12
Book / eBook (9) 9
Conference Proceeding (7) 7
Dissertation (2) 2
Magazine Article (2) 2
Reference (1) 1
Web Resource (1) 1
more...
Subjects Subjects
Subjects Subjects
X
Sort by Item Count (A-Z)
Filter by Count
sphingolipidoses (472) 472
humans (445) 445
research (163) 163
female (149) 149
male (131) 131
index medicus (119) 119
animals (111) 111
sphingolipidoses - metabolism (106) 106
medical research (99) 99
infant (97) 97
sphingolipidoses - pathology (97) 97
child, preschool (95) 95
medicine, experimental (94) 94
sphingolipidoses - genetics (88) 88
sphingolipidoses - diagnosis (86) 86
child (85) 85
reports (85) 85
enzymes (77) 77
genetic aspects (72) 72
adult (70) 70
biochemistry & molecular biology (62) 62
metachromatic leukodystrophy (61) 61
analysis (60) 60
sphingolipidoses - enzymology (60) 60
lipids (59) 59
adolescent (55) 55
care and treatment (54) 54
sphingolipids - metabolism (54) 54
genetics & heredity (53) 53
sphingolipids (53) 53
gene therapy (50) 50
genetic research (49) 49
mutation (43) 43
cell biology (40) 40
neurosciences (40) 40
physiological aspects (40) 40
transplantation (40) 40
nervous system diseases (39) 39
clinical neurology (38) 38
infant, newborn (38) 38
genes (36) 36
lysosomal storage diseases (36) 36
pregnancy (36) 36
cells, cultured (35) 35
diagnosis (35) 35
leukodystrophy, metachromatic - genetics (34) 34
development and progression (33) 33
metabolism (33) 33
pediatrics (33) 33
gaucher disease (32) 32
ceramide (31) 31
mice (31) 31
diagnosis, differential (30) 30
lysosomes - enzymology (30) 30
medicine, research & experimental (29) 29
leukodystrophy, metachromatic - diagnosis (28) 28
lysosomal storage disorders (28) 28
middle aged (28) 28
disease (27) 27
disorders (27) 27
prenatal diagnosis (27) 27
biochemistry (26) 26
lysosomes - metabolism (26) 26
sphingolipidoses - complications (26) 26
cerebroside-sulfatase - genetics (25) 25
brain - pathology (24) 24
fibroblasts - enzymology (24) 24
magnetic resonance imaging (24) 24
universities and colleges (24) 24
disease models, animal (23) 23
glycosphingolipids - metabolism (23) 23
health aspects (23) 23
leukodystrophy (23) 23
lipidoses (23) 23
enzyme replacement therapy (22) 22
gangliosides - metabolism (22) 22
gaucher-disease (22) 22
molecular sequence data (22) 22
hematopoietic stem cells (21) 21
phenotype (21) 21
fabry disease (20) 20
genetics (20) 20
liver - enzymology (20) 20
microscopy, electron (20) 20
pathology (20) 20
research article (20) 20
sphingolipidoses - therapy (20) 20
biology (19) 19
brain - metabolism (19) 19
ceramides - metabolism (19) 19
gaucher disease - metabolism (19) 19
leukodystrophy, metachromatic - pathology (19) 19
proteins (19) 19
sphingolipidoses - drug therapy (19) 19
fibroblasts - metabolism (18) 18
gangliosides (18) 18
gene mutations (18) 18
heterozygote (18) 18
mass spectrometry (18) 18
metabolism, inborn errors of (18) 18
more...
Library Location Library Location
Language Language
Language Language
X
Sort by Item Count (A-Z)
Filter by Count
English (711) 711
French (28) 28
German (25) 25
Japanese (18) 18
Russian (9) 9
Italian (6) 6
Polish (4) 4
Chinese (3) 3
Spanish (3) 3
Dutch (2) 2
Finnish (1) 1
Hungarian (1) 1
Norwegian (1) 1
Romanian (1) 1
Swedish (1) 1
Turkish (1) 1
more...
Publication Date Publication Date
Click on a bar to filter by decade
Slide to change publication date range


1. Sphingolipidoses and allied disorders
by Adachi, M and Schneck, L and Volk, B
1979, Annual research reviews., ISBN 0888310552
Sphingolipidoses | Lipids | Metabolism | Disorders
Book
Details down arrow
2. Full Text A view on sphingolipids and disease
by Kolter, Thomas
Chemistry and Physics of Lipids, ISSN 0009-3084, 2011, Volume 164, Issue 6, pp. 590 - 606
â–º Pathogenesis of sphingolipidoses is determined by the cell type-specific expression of sphingolipids and glycosphingolipids. â–º Sphingolipid biosynthesis is... 
Metabolic disease | Sphingolipidoses | Lipids | Sphingolipids | Gangliosides | CERAMIDE BIOSYNTHESIS | BIOCHEMISTRY & MOLECULAR BIOLOGY | SIALIC-ACID | ACTIVATOR PROTEINS | LYSOSOMAL STORAGE DISORDERS | INSULIN SENSITIVITY | SENSORY NEUROPATHY TYPE-1 | SKELETAL-MUSCLE | BIOPHYSICS | SERINE PALMITOYLTRANSFERASE | BIOACTIVE SPHINGOLIPIDS | ACID SPHINGOMYELINASE | Carbohydrate Sequence | Sphingolipidoses - chemically induced | Sphingolipidoses - pathology | Sphingolipids - biosynthesis | Sphingolipids - chemistry | Disease - genetics | Glycosphingolipids - biosynthesis | Humans | Molecular Sequence Data | Sphingolipids - metabolism | Sphingolipidoses - enzymology | Animals | Lysosomes - metabolism | Glycosphingolipids - metabolism | Sphingolipidoses - metabolism | Glycosphingolipids - chemistry | Drug resistance in microorganisms | Atherosclerosis
Journal Article
Details down arrow
Digital rights down arrow
loading Loading Rights
3. Sphingolipid biochemistry
by Kanfer, Julian N and Hakomori, Sen-itiroh
1983, ISBN 0306410923, Volume 3., xiv, 485
Sphingolipidoses | Metabolism | Glycosphingolipids
Book
Details down arrow
4. Full Text Mitochondrial Respiration Controls Lysosomal Function during Inflammatory T Cell Responses
by Baixauli, Francesc and Acín-Pérez, Rebeca and Villarroya-Beltrí, Carolina and Mazzeo, Carla and Nuñez-Andrade, Norman and Gabandé-Rodriguez, Enrique and Ledesma, Maria Dolores and Blázquez, Alberto and Martin, Miguel Angel and Falcón-Pérez, Juan Manuel and Redondo, Juan Miguel and Enríquez, Jose Antonio and Mittelbrunn, Maria
Cell Metabolism, ISSN 1550-4131, 09/2015, Volume 22, Issue 3, pp. 485 - 498
The endolysosomal system is critical for the maintenance of cellular homeostasis. However, how endolysosomal compartment is regulated by mitochondrial function... 
GLYCOLYSIS | LIPID-METABOLISM | BIOGENESIS | DNA | DISEASE | CALCIUM | ENDOCRINOLOGY & METABOLISM | STORAGE DISORDERS | AUTOPHAGY | DYSFUNCTION | TRANSCRIPTION FACTOR | CELL BIOLOGY | Sphingolipidoses - pathology | Lysosomes - genetics | Mitochondria - immunology | Mitochondrial Proteins - genetics | CD4-Positive T-Lymphocytes - pathology | CD4-Positive T-Lymphocytes - immunology | Cell Respiration | Lysosomal Storage Diseases - immunology | Mitochondria - genetics | T-Lymphocytes - metabolism | Gene Deletion | Lysosomes - pathology | Sphingolipidoses - immunology | T-Lymphocytes - pathology | Transcription Factors - immunology | DNA-Binding Proteins - immunology | CD4-Positive T-Lymphocytes - metabolism | Lysosomal Storage Diseases - genetics | Mitochondria - pathology | Transcription Factors - genetics | DNA-Binding Proteins - genetics | Lysosomal Storage Diseases - pathology | Sphingolipidoses - genetics | Animals | T-Lymphocytes - immunology | Mice | Immunity, Cellular | Lysosomes - immunology | Mitochondrial Proteins - immunology | Inflammation | Mitochondrial DNA | T cells | Cell differentiation | Analysis | Neurophysiology | autophagy | lysosome storage disorder | inflammation | mitochondria | T cell differentiation | glycolysis | TFEB | TFAM | sphingomyelin | metabolism | fatty acid oxidation
Journal Article
Details down arrow
Digital rights down arrow
loading Loading Rights
5. Full Text Fluid levity of the cell: Role of membrane lipid architecture in genetic sphingolipidoses
by D'Auria, Ludovic and Bongarzone, Ernesto R
Journal of Neuroscience Research, ISSN 0360-4012, 11/2016, Volume 94, Issue 11, pp. 1019 - 1024
Sphingolipidoses arise from inherited loss of function of key enzymes regulating the sphingolipid (SL) metabolism and the accumulation of large quantities of... 
lipid rafts | psychosine | Krabbe's disease | fluidity | sphingolipids | neurodegeneration | caveolin | CHOLESTEROL | CERAMIDE | NEUROSCIENCES | ORGANIZATION | MODEL MEMBRANES | DOMAIN FORMATION | RAFTS | SPHINGOMYELINASE | DISEASE | PROTEINS | Sphingolipidoses - pathology | Animals | Membrane Lipids - genetics | Humans | Membrane Fluidity - physiology | Membrane Lipids - deficiency | Sphingolipidoses - genetics | Sphingolipidoses | Physiological aspects | Lipids | Nervous system diseases | Index Medicus | Krabbe’s disease
Journal Article
Details down arrow
Digital rights down arrow
loading Loading Rights
6. Full Text OP1 – 2527: Phenotypic variation between siblings with metachromatic leukodystrophy
by Groeschel, S and Waibel, J and van Rappard, D.F and Kehrer, C and Böhringer, J and Schöls, L and Wolf, N and Krägeloh-Mann, I
European Journal of Paediatric Neurology, ISSN 1090-3798, 2015, Volume 19, pp. S1 - S1
Objective Metachromatic Leukodystrophy (MLD) is a rare autosomal-recessive disorder caused by mutations in the Arylsulfatase A gene. Although over 160... 
Pediatrics | Neurology | Sphingolipidoses | Analysis
Journal Article
Details down arrow
Digital rights down arrow
loading Loading Rights
7. Full Text The sphingolipid salvage pathway in ceramide metabolism and signaling
by Kitatani, Kazuyuki and Idkowiak-Baldys, Jolanta and Hannun, Yusuf A
Cellular Signalling, ISSN 0898-6568, 2008, Volume 20, Issue 6, pp. 1010 - 1018
Sphingolipids are important components of eukaryotic cells, many of which function as bioactive signaling molecules. Of these, ceramide is a central metabolite... 
Fumonisin B1 | Protein kinase C | Salvage pathway | Sphingosine | Ceramide | Sphingolipid | Ceramide signal | DIHYDROCERAMIDE DESATURASE | salvage pathway | PROTEIN-KINASE-C | DE-NOVO | sphingolipid | INDUCED APOPTOSIS | NOVO-SYNTHESIZED CERAMIDE | NECROSIS-FACTOR-ALPHA | ceramide signal | sphingosine | ADENOCARCINOMA CELL-LINE | CELL BIOLOGY | ceramide | SERINE PALMITOYLTRANSFERASE | ACID BETA-GLUCOSIDASE | SPHINGOSINE KINASE | fumonisin BI | protein kinase C | Sphingolipids - metabolism | Animals | Ceramides - metabolism | Signal Transduction | Sphingolipidoses - enzymology | Sphingolipidoses - genetics | Physiological aspects | Enzymes | Lipids | Metabolites | RNA | fumonisin B1
Journal Article
Details down arrow
Digital rights down arrow
loading Loading Rights
8. Practical enzymology of the sphingolipidoses
by Glew, Robert H and Peters, Stephen P
1977, Laboratory and research methods in biology and medicine ; v. 1., ISBN 9780845116500, Volume 1., xvi, 305
Sphingolipidoses | Glycosidase | Lysosomes | Diagnosis | Analysis
Book
Details down arrow
9. Sphingolipids and metabolic disease
by Cowart, Lauren Ashley
2011, Advances in experimental medicine and biology, ISBN 1461406498, Volume 721., xvii, 152
This book addresses not only the fundamentals of sphingolipid metabolism and analysis, but also the roles of sphingolipids in these disease processes. It... 
Sphingolipidoses | Sphingolipids | metabolism | pathology | Pathophysiology | Metabolic Diseases
Book
Details down arrow
10. Full Text DNA Nanostructures as Smart Drug-Delivery Vehicles and Molecular Devices
by Linko, Veikko and Ora, Ari and Kostiainen, Mauri A
Trends in Biotechnology, ISSN 0167-7799, 2015, Volume 33, Issue 10, pp. 586 - 594
DNA molecules can be assembled into custom predesigned shapes via hybridization of sequence-complementary domains. The folded structures have high spatial... 
Internal Medicine | self-assembly | biocomputing | cell uptake | immunostimulation | biosensing | DNA nanotechnology | enzymatic payloads | DNA origami | drug delivery | Cell uptake | Self-assembly | Immunostimulation | Biocomputing | Drug delivery | Biosensing | Enzymatic payloads | FOLDING DNA | ASSEMBLIES | NUCLEIC-ACID | BIOTECHNOLOGY & APPLIED MICROBIOLOGY | IN-VIVO | NANOSCALE SHAPES | ORIGAMI NANOSTRUCTURES | INTRACELLULAR DELIVERY | CELLULAR DELIVERY | SCAFFOLDS | CANCER-THERAPY | Sphingolipidoses - pathology | Drug Delivery Systems - instrumentation | Signal Transduction | Nanotechnology - methods | Humans | Nanotechnology - instrumentation | DNA - metabolism | Sphingolipidoses - therapy | Enzyme Replacement Therapy - methods | DNA - chemistry | Neoplasms - therapy | Base Pairing | Nanostructures - chemistry | Neoplasms - pathology | Nucleic Acid Conformation | Drug Delivery Systems - methods | Drugs | Drug delivery systems | DNA | Smart drugs and nutrients | Vehicles | Design | Proteins | Medical research | Ligands | Nanotechnology | Deoxyribonucleic acid--DNA | Cancer | Index Medicus
Journal Article
Details down arrow
Digital rights down arrow
loading Loading Rights
11. Full Text Sphingolipids and neuronal degeneration in lysosomal storage disorders
by Grassi, Sara and Chiricozzi, Elena and Mauri, Laura and Sonnino, Sandro and Prinetti, Alessandro
Journal of Neurochemistry, ISSN 0022-3042, 03/2019, Volume 148, Issue 5, pp. 600 - 611
Ceramide, sphingomyelin, and glycosphingolipids (both neutral and acidic) are characterized by the presence in the lipid moiety of an aliphatic base known as... 
gangliosides | sphingomyelin | sphingolipidoses | sphingolipids | lysosome | membrane fusion | EXOGENOUS GANGLIOSIDES | GM1 GANGLIOSIDE | ENRICHED MEMBRANE DOMAINS | INNER ESTER | BIOCHEMISTRY & MOLECULAR BIOLOGY | ANCHORED PROTEINS | PLASMA-MEMBRANE | NEUROSCIENCES | CEREBELLAR GRANULE CELLS | ENZYME REPLACEMENT | GANGLIOSIDE METABOLISM | SKIN FIBROBLASTS | Physiological aspects | Lipids | Neurons | Gangliosides | Sphingosine | Membrane proteins | Membranes | Hydrolase | Disorders | Lysosomes | Lysosomal storage diseases | Sphingolipids | Cell membranes | Accumulation | Aliphatic compounds | Substrates | Proteins | Glycosphingolipids | Storage | Plasma membranes | Ceramide | Sphingomyelin | Degeneration | Metabolic pathways
Journal Article
Details down arrow
Digital rights down arrow
loading Loading Rights
12. Full Text My journey into the world of sphingolipids and sphingolipidoses
by SANDHOFF, Konrad
Proceedings of the Japan Academy, Series B, ISSN 0386-2208, 2012, Volume 88, Issue 10, pp. 554 - 582
Analysis of lipid storage in postmortem brains of patients with amaurotic idiocy led to the recognition of five lysosomal ganglioside storage diseases and... 
endocytosis of membrane lipids | lipid storage diseases | lysosomal digestion | lipids regulate endolysosomal proteins | ganglioside metabolism | lipid binding proteins | Lipid storage diseases | Ganglioside metabolism | Lipids regulate endolysosomal proteins | Endocytosis of membrane lipids | Lysosomal digestion | Lipid binding proteins | MULTIDISCIPLINARY SCIENCES | BETA-HEXOSAMINIDASE-A | HUMAN GM2-ACTIVATOR PROTEIN | GM2 ACTIVATOR PROTEIN | THIN-LAYER CHROMATOGRAPHY | INFANTILE AMAUROTIC IDIOCY | NIEMANN-PICK-DISEASE | TRICYCLIC ANTIDEPRESSANT DESIPRAMINE | HUMAN ACID SPHINGOMYELINASE | MANNOSE 6-PHOSPHATE RECEPTOR | TAY-SACHS DISEASE | Sphingolipidoses - pathology | Sphingolipids - biosynthesis | Sphingolipid Activator Proteins - chemistry | Humans | Sphingolipid Activator Proteins - metabolism | Lysosomes - enzymology | Sphingolipids - metabolism | Sphingolipidoses - enzymology | Sphingolipidoses - genetics | Brain - metabolism | Endocytosis | Animals | Lysosomes - metabolism | Brain - pathology | Sphingolipidoses - metabolism | Neurochemistry | Brain | Enzymes | Lipids | Biosynthesis | Review
Journal Article
Details down arrow
Digital rights down arrow
loading Loading Rights