Systemic ceramide accumulation leads to severe and varied pathological consequences.

scientific article published on 16 May 2013

Systemic ceramide accumulation leads to severe and varied pathological consequences. is …
instance of (P31):
scholarly articleQ13442814

External links are
P356DOI10.1002/EMMM.201202301
P932PMC publication ID3779446
P698PubMed publication ID23681708
P5875ResearchGate publication ID236916193

P50authorEdward H SchuchmanQ92756224
Calogera M. SimonaroQ111458084
Gilbert G PrivéQ56458223
Razq HakemQ58381166
Patricia V. TurnerQ59158717
Gemma FabriasQ62513752
P2093author name stringThierry Levade
Jeffrey A Medin
Josefina Casas
Stéphane Carpentier
James C M Wang
Shaalee Dworski
Virginie Garcia
Kevin N Kirouac
Zi Jian Xiong
Abdulfatah M Alayoubi
Bryan C Y Au
Mathilde J Exertier
Samah El-Ghamrasni
P2860cites workI-TASSER server for protein 3D structure predictionQ21284202
Central role of ceramide biosynthesis in body weight regulation, energy metabolism, and the metabolic syndromeQ24648112
Structures of the human ceramide activator protein saposin DQ27650509
Structures of an isopenicillin N converting Ntn-hydrolase reveal different catalytic roles for the active site residues of precursor and mature enzymeQ27660186
The human acid ceramidase gene (ASAH): structure, chromosomal location, mutation analysis, and expressionQ28141241
Farber's disease. Light and electron microscopic study of the eyeQ70080392
Farber disease: pathologic diagnosis in sibs with phenotypic variabilityQ70388571
Neurodegenerative course in ceramidase deficiency (Farber disease) correlates with the residual lysosomal ceramide turnover in cultured living patient cellsQ71367362
High level monocyte chemoattractant protein-1 expression in transgenic mice increases their susceptibility to intracellular pathogensQ71829036
Percutaneous intravenous injection in neonatal miceQ77993909
Inhibition of MCP-1/CCR2 pathway ameliorates the development of diabetic nephropathyQ80613413
Editorial [hot topic: Therapeutic targeting of the sphingolipid "biostat" in hematologic malignancies (guest editors: Thomas P. Loughran and Hong-Gang Wang)]Q82170528
Ceramide kinase deficiency improves diet-induced obesity and insulin resistanceQ83783188
Molecular analysis of acid ceramidase deficiency in patients with Farber diseaseQ28204074
MCP-1 contributes to macrophage infiltration into adipose tissue, insulin resistance, and hepatic steatosis in obesityQ29614283
Controlled recruitment of monocytes and macrophages to specific organs through transgenic expression of monocyte chemoattractant protein-1.Q34290308
Determination of ceramides and diglycerides by the diglyceride kinase assayQ34470701
The therapeutic potential of modulating the ceramide/sphingomyelin pathwayQ34717833
Genistein stimulates MCF-7 breast cancer cell growth by inducing acid ceramidase (ASAH1) gene expressionQ35010927
Autologous transplantation of lentivector/acid ceramidase-transduced hematopoietic cells in nonhuman primatesQ35161467
The PDL1-PD1 axis converts human TH1 cells into regulatory T cellsQ35607698
Sphingosine-1-phosphate signaling and its role in diseaseQ35661485
Biologically active sphingolipids in cancer pathogenesis and treatmentQ35852313
Farber disease: clinical presentation, pathogenesis and a new approach to treatmentQ35894517
Acid ceramidase-mediated production of sphingosine 1-phosphate promotes prostate cancer invasion through upregulation of cathepsin BQ36063053
Acid ceramidase and human diseaseQ36634467
In vivo delivery of human acid ceramidase via cord blood transplantation and direct injection of lentivirus as novel treatment approaches for Farber diseaseQ37038201
Construction of conditional acid ceramidase knockout mice and in vivo effects on oocyte development and fertilityQ37091180
Bioluminescent imaging of a marking transgene and correction of Fabry mice by neonatal injection of recombinant lentiviral vectorsQ37695619
Altered adipose and plasma sphingolipid metabolism in obesity: a potential mechanism for cardiovascular and metabolic riskQ38310300
Autoproteolytic cleavage and activation of human acid ceramidaseQ39262060
Farber's disease in two siblings, sural nerve and subcutaneous biopsies by light and electron microscopyQ39593663
Methods for studying the cellular response to DNA damage: influence of the Mre11 complex on chromosome metabolism.Q40263773
Retrovirus-mediated correction of the metabolic defect in cultured Farber disease cells.Q40809927
In situ assay of acid sphingomyelinase and ceramidase based on LDL-mediated lysosomal targeting of ceramide-labeled sphingomyelin.Q41146655
Farber's disease without central nervous system involvement: bone-marrow transplantation provides a promising new approachQ42955175
The role of ceramide in receptor- and stress-induced apoptosis studied in acidic ceramidase-deficient Farber disease cells.Q43771751
Insertional mutagenesis of the mouse acid ceramidase gene leads to early embryonic lethality in homozygotes and progressive lipid storage disease in heterozygotesQ43877896
Synthesis and biological activity of a novel inhibitor of dihydroceramide desaturaseQ46781276
Decreased lesion formation in CCR2-/- mice reveals a role for chemokines in the initiation of atherosclerosisQ46897070
Ceramidase Deficiency in Farber's Disease (Lipogranulomatosis)Q48710659
Human acid ceramidase gene: novel mutations in Farber disease.Q50336005
Farber lipogranulomatosis type 1--late presentation and early death in a Croatian boy with a novel homozygous ASAH1 mutation.Q50571349
First case of surgical treatment of Farber's disease.Q51535429
Bone marrow transplantation for infantile ceramidase deficiency (Farber disease).Q52165621
Acid ceramidase is a novel factor required for early embryo survival.Q54559245
Phenotypic variability in siblings with Farber diseaseQ57162604
P4510describes a project that usesImageQuantQ112270642
P433issue6
P407language of work or nameEnglishQ1860
P921main subjectceramidesQ424213
P304page(s)827-842
P577publication date2013-05-16
P1433published inEMBO Molecular MedicineQ15817279
P1476titleSystemic ceramide accumulation leads to severe and varied pathological consequences
P478volume5

Reverse relations

cites work (P2860)
Q35472380A ceramide analogue stimulates dendritic cells to promote T cell responses upon virus infections
Q95660487ASAH1 pathogenic variants associated with acid ceramidase deficiency (ACD): Farber disease and spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME)
Q64042504Acid Ceramidase Deficiency in Mice Leads to Severe Ocular Pathology and Visual Impairment
Q48245644Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities
Q38787556Acid Ceramidase Deficiency is characterized by a unique plasma cytokine and ceramide profile that is altered by therapy.
Q89944674Acid Ceramidase Depletion Impairs Neuronal Survival and Induces Morphological Defects in Neurites Associated with Altered Gene Transcription and Sphingolipid Content
Q91966157Acid Sphingomyelinase Deficiency Ameliorates Farber Disease
Q90370648Acid ceramidase deficiency: Farber disease and SMA-PME
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Q36756813Anti-inflammatory Therapy With Simvastatin Improves Neuroinflammation and CNS Function in a Mouse Model of Metachromatic Leukodystrophy
Q41094126C26-Ceramide as highly sensitive biomarker for the diagnosis of Farber Disease
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Q47833651Deletion of MCP-1 Impedes Pathogenesis of Acid Ceramidase Deficiency
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Q91595378Farber disease: report of three cases with joint involvement mimicking juvenile idiopathic arthritis
Q37188548Farber disease: understanding a fatal childhood disorder and dissecting ceramide biology.
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Q36405917Glycosphingolipids and cell death: one aim, many ways
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Q92667796Hepatic pathology and altered gene transcription in a murine model of acid ceramidase deficiency
Q38885436Increased acid ceramidase expression depends on upregulation of androgen-dependent deubiquitinases, USP2, in a human prostate cancer cell line, LNCaP.
Q38793821Inhibiting glucosylceramide synthase exacerbates cisplatin-induced acute kidney injury
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Q47673905Loss of acid ceramidase in myeloid cells suppresses intestinal neutrophil recruitment.
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Q50079745Sphingolipid pathway enzymes modulate cell fate and immune responses
Q55248134Structural basis for the activation of acid ceramidase.

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