Ceramide accumulation is associated with increased apoptotic cell death in cultured fibroblasts of sphingolipid activator protein-deficient mouse but not in fibroblasts of patients with Farber disease

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Ceramide accumulation is associated with increased apoptotic cell death in cultured fibroblasts of sphingolipid activator protein-deficient mouse but not in fibroblasts of patients with Farber disease is …
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scholarly articleQ13442814

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P6179Dimensions Publication ID1014966012
P356DOI10.1023/A:1005590316064
P698PubMed publication ID10399097

P2093author name stringK Suzuki
N Fujita
M T Vanier
H Nakayasu
J Tohyama
Y Oya
T Ezoe
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Role of ceramide as a lipid mediator of 1 alpha,25-dihydroxyvitamin D3-induced HL-60 cell differentiationQ41721364
Additional biochemical findings in a patient and fetal sibling with a genetic defect in the sphingolipid activator protein (SAP) precursor, prosaposin. Evidence for a deficiency in SAP-1 and for a normal lysosomal neuraminidaseQ42071359
Ceramide is a competitive inhibitor of diacylglycerol kinase in vitro and in intact human leukemia (HL-60) cellsQ42450545
Acid sphingomyelinase-deficient human lymphoblasts and mice are defective in radiation-induced apoptosis.Q42555356
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Metabolism of cerebroside sulphate and subcellular distribution of its metabolites in cultured skin fibroblasts derived from controls, metachromatic leukodystrophy, globoid cell leukodystrophy and Farber diseaseQ44736843
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Programmed cell death in cortical chick embryo astrocytes is associated with activation of protein kinase PK60 and ceramide formation.Q48568860
Ceramide protects hippocampal neurons against excitotoxic and oxidative insults, and amyloid beta-peptide toxicityQ49155965
Ceramide induces apoptosis in cultured mesencephalic neuronsQ49156263
Apoptosis involved in density-dependent regulation of rat fibroblastic 3Y1 cell culture.Q50533424
Protection by prosaposin against ischemia-induced learning disability and neuronal loss.Q52213537
Targeted disruption of the mouse sphingolipid activator protein gene: a complex phenotype, including severe leukodystrophy and wide-spread storage of multiple sphingolipids.Q52520885
The gene coding for a sphingolipid activator protein, SAP-1, is on human chromosome 10Q70072985
Metabolism of ceramide-containing endocytotic vesicles in human diploid fibroblastsQ70288563
Programmed cell death induced by ceramideQ70619078
Role of lysosomal acid ceramidase in the metabolism of ceramide in human skin fibroblastsQ70862552
(1S,2R)-D-erythro-2-(N-myristoylamino)-1-phenyl-1-propanol as an inhibitor of ceramidaseQ71119928
Opposite effects of tumor necrosis factor alpha on the sphingomyelin-ceramide pathway in two myeloid leukemia cell lines: role of transverse sphingomyelin distribution in the plasma membraneQ71228956
Farber's lipogranulomatosis. Report of a case and demonstration of an excess of free ceramide and gangliosideQ71359805
Neurodegenerative course in ceramidase deficiency (Farber disease) correlates with the residual lysosomal ceramide turnover in cultured living patient cellsQ71367362
Prosaposin facilitates sciatic nerve regeneration in vivoQ71464564
A family with combined Farber and Sandhoff, isolated Sandhoff and isolated fetal Farber disease: postnatal exclusion and prenatal diagnosis of Farber disease using lipid loading tests on intact cultured cellsQ71814795
Ceramide prevents neuronal programmed cell death induced by nerve growth factor deprivationQ72334860
Sphingolipid activator protein D (sap-D) stimulates the lysosomal degradation of ceramide in vivoQ72425473
Prosaposin deficiency: further characterization of the sphingolipid activator protein-deficient sibs. Multiple glycolipid elevations (including lactosylceramidosis), partial enzyme deficiencies and ultrastructure of the skin in this generalized sphiQ72921443
Loss of ceramide production confers resistance to radiation-induced apoptosisQ73221177
Natural ceramide is unable to escape the lysosome, in contrast to a fluorescent analogueQ74582933
P433issue5
P921main subjectsphingolipidsQ410395
ceramidesQ424213
apoptotic processQ14599311
P304page(s)649-662
P577publication date1999-06-01
P1433published inJournal of Inherited Metabolic DiseaseQ6295359
P1476titleCeramide accumulation is associated with increased apoptotic cell death in cultured fibroblasts of sphingolipid activator protein-deficient mouse but not in fibroblasts of patients with Farber disease
P478volume22

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cites work (P2860)
Q33629581An inverse relationship between ceramide synthesis and clinical severity in patients with psoriasis
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Q43717679Psychosine is as potent an inducer of cell death as C6-ceramide in cultured fibroblasts and in MOCH-1 cells
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Q36995806The Effect of Gromwell (Lithospermum erythrorhizon) Extract on the Stratum Corneum Hydration and Ceramides Content in Atopic Dermatitis Patients

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