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BBA - Molecular and Cell Biology of Lipids, ISSN 1388-1981, 03/2014, Volume 1841, Issue 3, pp. 441 - 452
The epidermal permeability barrier of mammalian skin is localized in the stratum corneum. Corneocytes are embedded in an extracellular, highly ordered lipid... 
Ceramide | Sphingomyelin | Glucosylceramide | Stratum corneum | Ultra long chain fatty acid | Acylceramide | Enzymes | Unsaturated fatty acids | Hydroxylation | Physiological aspects | Ceramides | Skin | Permeability | Plant lipids | Glutamate | Protein binding
Journal Article
Biological chemistry, ISSN 1431-6730, 08/2019
Drug-induced phospholipidosis is a lysosomal storage disorder characterized by excessive accumulation of phospholipids. Its cellular mechanism is still not... 
Journal Article
Methods in Molecular Biology, ISSN 1064-3745, 2018, Volume 1804, pp. 97 - 141
Gangliosides are sialic acid containing glycosphingolipids, which are abundant in mammalian brain tissue. Several fatal human diseases are caused by defects in... 
Gangliosidosis | Membranes | Sialic acid | Glycosphingolipids | Luminal vesicles | Ceramide | Biosynthesis | Catabolism | Bis(monoacylglycero)phosphate
Journal Article
Journal of lipid research, ISSN 0022-2275, 06/2019, Volume 60, Issue 6, pp. 1099 - 1111
The catabolism of ganglioside GM2 is dependent on three gene products. Mutations in any of these genes result in a different type of GM2 gangliosidosis... 
Journal Article
Molecular genetics and metabolism, ISSN 1096-7192, 05/2019
The catabolism of ganglioside GM2 is dependent on the lysosomal enzyme β-hexosaminidase A and a supporting lipid transfer protein, the GM2 activator protein. A... 
Journal Article
Annual Review of Biochemistry, ISSN 0066-4154, 6/2019, Volume 88, Issue 1, pp. 461 - 485
Journal Article
Journal of Lipid Research, ISSN 0022-2275, 2017, Volume 58, Issue 3, pp. 563 - 577
Glucosylceramide (GlcCer) is the primary storage lipid in the lysosomes of Gaucher patients and a secondary one in Niemann-Pick disease types A, B, and C. The... 
Lysophospholipids | Gaucher disease | Anionic phospholipids | Cationic lipids | Electrostatic interaction | Sphingosine | Sphingomyelin | Acylglycerols | Fatty acids | Cholesterol | GH3 PITUITARY-CELLS | fatty acids | SPHINGOLIPID ACTIVATOR PROTEINS | BIOCHEMISTRY & MOLECULAR BIOLOGY | anionic phospholipids | sphingosine | GAUCHER-DISEASE | CHOLESTEROL TRAFFICKING | SAPOSIN-C | NIEMANN-PICK-DISEASE | UNILAMELLAR VESICLES | TRICYCLIC ANTIDEPRESSANT DESIPRAMINE | lysophospholipids | cationic lipids | cholesterol | sphingomyelin | electrostatic interaction | ACID SPHINGOMYELINASE ACTIVITY | acylglycerols | EXTRACTION CAPACITY | Lysophospholipids - metabolism | Glucosylceramidase - genetics | Gaucher Disease - metabolism | Genetic Association Studies | Humans | Lysosomes - enzymology | Glucosylceramidase - metabolism | Gaucher Disease - pathology | Niemann-Pick Diseases - metabolism | Cholesterol - metabolism | Niemann-Pick Diseases - pathology | Monoglycerides - metabolism | Hydrolysis | Saposins - metabolism | Gaucher Disease - genetics | Lipid Metabolism - genetics | Glucosylceramides - metabolism | Niemann-Pick Diseases - genetics | Niemann-Pick disease | Diacylglycerol | Lysosomes | Lipids | Phospholipids | pH effects | Electrostatic properties | Sphinganine | Ceramide | Bone morphogenetic proteins | Glucosylceramidase | Genotypes
Journal Article
Journal Article
Journal of Lipid Research, ISSN 0022-2275, 10/2015, Volume 56, Issue 10, pp. 1861 - 1879
Journal Article
Journal Article
Journal of Lipid Research, ISSN 0022-2275, 09/2015, Volume 56, Issue 9, pp. 1747 - 1761
Ganglioside GM2 is the major lysosomal storage compound of Tay-Sachs disease. It also accumulates in Niemann-Pick disease types A and B with primary storage of... 
Supplementary lipid transfer protein | Ceramide | Sphingomyelin | Hexahistidine-tag | Endosomal | Bis(monoacylglycero)phosphate | Cholesterol | Lysosomal lipids | CHOLESTEROL TRANSFER | BIOCHEMISTRY & MOLECULAR BIOLOGY | BETA-HEXOSAMINIDASE-A | HUMAN GM2-ACTIVATOR PROTEIN | endosomal | lipid transfer protein | RAT-LIVER LYSOSOMES | C2 PROTEIN | IMMUNOELECTRON MICROSCOPY | bis(monoacylglycero)phosphate | NIEMANN-PICK-DISEASE | ceramide | hexahistidine-tag | MOBILIZES LIPIDS | sphingomyelin | cholesterol | lysosomal lipids | ACID SPHINGOMYELINASE ACTIVITY | EXTRACTION CAPACITY | Surface Plasmon Resonance | Lysophospholipids - administration & dosage | Ceramides - metabolism | G(M2) Activator Protein - metabolism | Sphingomyelins - metabolism | Hydrolysis - drug effects | Membrane Lipids - genetics | G(M2) Activator Protein - genetics | Humans | beta-Hexosaminidase alpha Chain - metabolism | Monoglycerides - administration & dosage | Niemann-Pick Diseases - metabolism | Cholesterol - metabolism | Niemann-Pick Diseases - pathology | Cholesterol - genetics | Tay-Sachs Disease - genetics | G(M2) Ganglioside - metabolism | HEK293 Cells | Tay-Sachs Disease - metabolism | Fluorescence Resonance Energy Transfer | Membrane Lipids - metabolism | Liposomes - metabolism | Tay-Sachs Disease - pathology | Niemann-Pick Diseases - genetics
Journal Article
Cell Reports, ISSN 2211-1247, 01/2018, Volume 22, Issue 4, pp. 967 - 978
Maintenance of metabolic homeostasis requires adaption of gene regulation to the cellular energy state via transcriptional regulators. Here, we identify a role... 
transcription factors | lipid sensing | homeodomain | lipid metabolism | ceramide synthase | peroxisome | energy homeostasis | genomic engineering | BETA-OXIDATION | FAT-BODY | HOMEOSTASIS | METABOLISM | INSULIN-RESISTANCE | HOMEODOMAIN PROTEINS | DROSOPHILA | LAG1P MOTIF | FAMILY | REGION | CELL BIOLOGY
Journal Article