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

No abstract is available for this paper.

• Publication year

  1999

• Venue

  Journal of Inherited Metabolic Disease

• Publication date

  1999-06-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1023/A:1005590316064PMID 10399097
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• The gene coding for a sphingolipid activator protein, SAP-1, is on human chromosome 10

  2004cited by this paper
• The Metabolic and Molecular Bases of Inherited Disease

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• Programmed Cell Death in Cortical Chick Embryo Astrocytes Is Associated with Activation of Protein Kinase PK60 and Ceramide Formation

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• Cell death prevention, mitogen-activated protein kinase stimulation, and increased sulfatide concentrations in Schwann cells and oligodendrocytes by prosaposin and prosaptides.

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• Loss of ceramide production confers resistance to radiation-induced apoptosis.

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• Turnover of endogenous ceramide in cultured normal and Farber fibroblasts.

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• (1S,2R)-D-erythro-2-(N-Myristoylamino)-1-phenyl-1-propanol as an Inhibitor of Ceramidase (*)

  1996cited by this paper
• Daunorubicin‐induced apoptosis: triggering of ceramide generation through sphingomyelin hydrolysis.

  1996cited by this paper
• Ceramide Protects Hippocampal Neurons Against Excitotoxic and Oxidative Insults, and Amyloid β‐Peptide Toxicity

  1996cited by this paper
• Staurosporine Induces Programmed Cell Death in Embryonic Neurons and Activation of the Ceramide Pathway

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• Apoptosis involved in density-dependent regulation of rat fibroblastic 3Y1 cell culture.

  1996cited by this paper
• Acid sphingomyelinase-deficient human lymphoblasts and mice are defective in radiation-induced apoptosis.

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• Targeted disruption of the mouse sphingolipid activator protein gene: a complex phenotype, including severe leukodystrophy and wide-spread storage of multiple sphingolipids.

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• Tumor cell resistance to apoptosis due to a defect in the activation of sphingomyelinase and the 24 kDa apoptotic protease (AP24)

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• Ceramide Induces Apoptosis in Cultured Mesencephalic Neurons

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• Prosaposin Facilitates Sciatic Nerve Regeneration In Vivo

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• 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 membrane.

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• Role of Ceramide in Cellular Senescence (*)

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• 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 cells

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• Ceramide Prevents Neuronal Programmed Cell Death Induced by Nerve Growth Factor Deprivation

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• Identification of the neurotrophic factor sequence of prosaposin

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• Ceramide synthase mediates daunorubicin-induced apoptosis: an alternative mechanism for generating death signals.

  1995cited by this paper
• Neurodegenerative course in ceramidase deficiency (Farber disease) correlates with the residual lysosomal ceramide turnover in cultured living patient cells.

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• Apoptotic signaling through CD95 (Fas/Apo-1) activates an acidic sphingomyelinase

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• Identification of prosaposin as a neurotrophic factor.

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• Ionizing radiation acts on cellular membranes to generate ceramide and initiate apoptosis

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• Induction of apoptotic DNA damage and cell death by activation of the sphingomyelin pathway.

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• Sphingolipid activator protein D (sap-D) stimulates the lysosomal degradation of ceramide in vivo.

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• Protection by prosaposin against ischemia-induced learning disability and neuronal loss.

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• Prosaposin deficiency: further characterization of the sphingolipid activator protein-deficient sibs

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• Simultaneous deficiency of sphingolipid activator proteins 1 and 2 is caused by a mutation in the initiation codon of their common gene.

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• Modulation of cell growth and differentiation by ceramide

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• 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 neuraminidase.

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• Ceramide is a competitive inhibitor of diacylglycerol kinase in vitro and in intact human leukemia (HL-60) cells.

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• Identification of sphingomyelin turnover as an effector mechanism for the action of tumor necrosis factor alpha and gamma-interferon. Specific role in cell differentiation.

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• Role of ceramide as a lipid mediator of 1 alpha,25-dihydroxyvitamin D3-induced HL-60 cell differentiation.

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• Sphingolipid activator protein deficiency in a 16-week-old atypical Gaucher disease patient and his fetal sibling: Biochemical signs of combined sphingolipidoses

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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 disease

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• Assignment of the gene for human sphingolipid activator protein-2 (SAP-2) to chromosome 10.

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• Genetic analysis of control of proliferation in fibroblastic cells in culture. II. Alteration in proliferative and survival phenotypes in a set of temperature-sensitive mutants of rat 3Y1 cells after infection or transformation with simian virus 40

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• Genetic analysis of control of proliferation in fibroblastic cells in culture. I. Isolation and characterization of mutants temperature-sensitive for proliferation or survival of untransformed diploid rat cell line 3Y1

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• Diagnosis of metachromatic leukodystrophy, Krabbe disease, and Farber disease after uptake of fatty acid-labeled cerebroside sulfate into cultured skin fibroblasts.

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• Role of lysosomal acid ceramidase in the metabolism of ceramide in human skin fibroblasts.

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• Farber's lipogranulomatosis: Report of a case and demonstration of an excess of free ceramide and ganglioside

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• Ceramide and Ganglioside Accumulation in Farber's Lipogranulomatosis.∗

  1967cited by this paper

• A Rationale for Hypoxic and Chemical Conditioning in Huntington’s Disease

  2021cites this paper
• Acid ceramidase deficiency: Farber lipogranulomatosis, spinal muscular atrophy associated with progressive myoclonic epilepsy and peripheral osteolysis

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• The Cardiac Lipidome in Models of Cardiovascular Disease

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• Extracellular Fatty Acids Are the Major Contributor to Lipid Synthesis in Prostate Cancer

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• Sphingolipid Metabolism: A New Therapeutic Opportunity for Brain Degenerative Disorders

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• Heterogeneity of fatty acid metabolism in breast cancer cells underlies differential sensitivity to palmitate‐induced apoptosis

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• Acid Ceramidase Deficiency: Farber Lipogranulomatosis and Spinal Muscular Atrophy Associated with Progressive Myoclonic Epilepsy

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• Dietary effect of red ginseng extracts mixed with torilis fructus and corni fructus on the epidermal levels of ceramides and ceramide related enzyme proteins in uv-induced hairless mice

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• The Effect of Gromwell (Lithospermum erythrorhizon) Extract on the Stratum Corneum Hydration and Ceramides Content in Atopic Dermatitis Patients.

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• Retrovirus-mediated Gene Therapy For Farber Disease

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• 실크단백질의 식이 공급이 아토피 피부염 동물 모델 NC/Nga Mice 피부의 세라마이드 함량 및 관련인자 발현에 미치는 영향

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• An Inverse Relationship Between Ceramide Synthesis and Clinical Severity in Patients with Psoriasis

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• Mutation in saposin D domain of sphingolipid activator protein gene causes urinary system defects and cerebellar Purkinje cell degeneration with accumulation of hydroxy fatty acid-containing ceramide in mouse.

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• Glycosphingolipidoses: Beyond the enzymatic defect

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• (cid:1) -Linolenic Acid in Borage Oil Reverses Epidermal Hyperproliferation in Guinea Pigs 1

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• Psychosine Is as Potent an Inducer of Cell Death as C6-Ceramide in Cultured Fibroblasts and in MOCH-1 Cells

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• Lysosomes

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