scholarly article | Q13442814 |
P50 | author | Edward H Schuchman | Q92756224 |
Calogera M. Simonaro | Q111458084 | ||
Gilbert G Privé | Q56458223 | ||
Razq Hakem | Q58381166 | ||
Patricia V. Turner | Q59158717 | ||
Gemma Fabrias | Q62513752 | ||
P2093 | author name string | Thierry 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 | |||
P2860 | cites work | I-TASSER server for protein 3D structure prediction | Q21284202 |
Central role of ceramide biosynthesis in body weight regulation, energy metabolism, and the metabolic syndrome | Q24648112 | ||
Structures of the human ceramide activator protein saposin D | Q27650509 | ||
Structures of an isopenicillin N converting Ntn-hydrolase reveal different catalytic roles for the active site residues of precursor and mature enzyme | Q27660186 | ||
The human acid ceramidase gene (ASAH): structure, chromosomal location, mutation analysis, and expression | Q28141241 | ||
Farber's disease. Light and electron microscopic study of the eye | Q70080392 | ||
Farber disease: pathologic diagnosis in sibs with phenotypic variability | Q70388571 | ||
Neurodegenerative course in ceramidase deficiency (Farber disease) correlates with the residual lysosomal ceramide turnover in cultured living patient cells | Q71367362 | ||
High level monocyte chemoattractant protein-1 expression in transgenic mice increases their susceptibility to intracellular pathogens | Q71829036 | ||
Percutaneous intravenous injection in neonatal mice | Q77993909 | ||
Inhibition of MCP-1/CCR2 pathway ameliorates the development of diabetic nephropathy | Q80613413 | ||
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 resistance | Q83783188 | ||
Molecular analysis of acid ceramidase deficiency in patients with Farber disease | Q28204074 | ||
MCP-1 contributes to macrophage infiltration into adipose tissue, insulin resistance, and hepatic steatosis in obesity | Q29614283 | ||
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 assay | Q34470701 | ||
The therapeutic potential of modulating the ceramide/sphingomyelin pathway | Q34717833 | ||
Genistein stimulates MCF-7 breast cancer cell growth by inducing acid ceramidase (ASAH1) gene expression | Q35010927 | ||
Autologous transplantation of lentivector/acid ceramidase-transduced hematopoietic cells in nonhuman primates | Q35161467 | ||
The PDL1-PD1 axis converts human TH1 cells into regulatory T cells | Q35607698 | ||
Sphingosine-1-phosphate signaling and its role in disease | Q35661485 | ||
Biologically active sphingolipids in cancer pathogenesis and treatment | Q35852313 | ||
Farber disease: clinical presentation, pathogenesis and a new approach to treatment | Q35894517 | ||
Acid ceramidase-mediated production of sphingosine 1-phosphate promotes prostate cancer invasion through upregulation of cathepsin B | Q36063053 | ||
Acid ceramidase and human disease | Q36634467 | ||
In vivo delivery of human acid ceramidase via cord blood transplantation and direct injection of lentivirus as novel treatment approaches for Farber disease | Q37038201 | ||
Construction of conditional acid ceramidase knockout mice and in vivo effects on oocyte development and fertility | Q37091180 | ||
Bioluminescent imaging of a marking transgene and correction of Fabry mice by neonatal injection of recombinant lentiviral vectors | Q37695619 | ||
Altered adipose and plasma sphingolipid metabolism in obesity: a potential mechanism for cardiovascular and metabolic risk | Q38310300 | ||
Autoproteolytic cleavage and activation of human acid ceramidase | Q39262060 | ||
Farber's disease in two siblings, sural nerve and subcutaneous biopsies by light and electron microscopy | Q39593663 | ||
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 approach | Q42955175 | ||
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 heterozygotes | Q43877896 | ||
Synthesis and biological activity of a novel inhibitor of dihydroceramide desaturase | Q46781276 | ||
Decreased lesion formation in CCR2-/- mice reveals a role for chemokines in the initiation of atherosclerosis | Q46897070 | ||
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 disease | Q57162604 | ||
P4510 | describes a project that uses | ImageQuant | Q112270642 |
P433 | issue | 6 | |
P407 | language of work or name | English | Q1860 |
P921 | main subject | ceramides | Q424213 |
P304 | page(s) | 827-842 | |
P577 | publication date | 2013-05-16 | |
P1433 | published in | EMBO Molecular Medicine | Q15817279 |
P1476 | title | Systemic ceramide accumulation leads to severe and varied pathological consequences | |
P478 | volume | 5 |
Q35472380 | A ceramide analogue stimulates dendritic cells to promote T cell responses upon virus infections |
Q95660487 | ASAH1 pathogenic variants associated with acid ceramidase deficiency (ACD): Farber disease and spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME) |
Q64042504 | Acid Ceramidase Deficiency in Mice Leads to Severe Ocular Pathology and Visual Impairment |
Q48245644 | Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities |
Q38787556 | Acid Ceramidase Deficiency is characterized by a unique plasma cytokine and ceramide profile that is altered by therapy. |
Q89944674 | Acid Ceramidase Depletion Impairs Neuronal Survival and Induces Morphological Defects in Neurites Associated with Altered Gene Transcription and Sphingolipid Content |
Q91966157 | Acid Sphingomyelinase Deficiency Ameliorates Farber Disease |
Q90370648 | Acid ceramidase deficiency: Farber disease and SMA-PME |
Q27310161 | Alkaline Ceramidase 3 Deficiency Results in Purkinje Cell Degeneration and Cerebellar Ataxia Due to Dyshomeostasis of Sphingolipids in the Brain |
Q36756813 | Anti-inflammatory Therapy With Simvastatin Improves Neuroinflammation and CNS Function in a Mouse Model of Metachromatic Leukodystrophy |
Q41094126 | C26-Ceramide as highly sensitive biomarker for the diagnosis of Farber Disease |
Q47372850 | Chronic lung injury and impaired pulmonary function in a mouse model of acid ceramidase deficiency |
Q47833651 | Deletion of MCP-1 Impedes Pathogenesis of Acid Ceramidase Deficiency |
Q37682583 | Enzyme replacement therapy for Farber disease: Proof-of-concept studies in cells and mice |
Q91595378 | Farber disease: report of three cases with joint involvement mimicking juvenile idiopathic arthritis |
Q37188548 | Farber disease: understanding a fatal childhood disorder and dissecting ceramide biology. |
Q90132216 | Genetic ablation of acid ceramidase in Krabbe disease confirms the psychosine hypothesis and identifies a new therapeutic target |
Q36405917 | Glycosphingolipids and cell death: one aim, many ways |
Q93029687 | Hematopoietic stem cell transplant does not prevent neurological deterioration in infants with Farber disease: Case report and literature review |
Q92667796 | Hepatic pathology and altered gene transcription in a murine model of acid ceramidase deficiency |
Q38885436 | Increased acid ceramidase expression depends on upregulation of androgen-dependent deubiquitinases, USP2, in a human prostate cancer cell line, LNCaP. |
Q38793821 | Inhibiting glucosylceramide synthase exacerbates cisplatin-induced acute kidney injury |
Q47102959 | Lipid Involvement in Neurodegenerative Diseases of the Motor System: Insights from Lysosomal Storage Diseases |
Q47673905 | Loss of acid ceramidase in myeloid cells suppresses intestinal neutrophil recruitment. |
Q42573873 | Markedly perturbed hematopoiesis in acid ceramidase deficient mice |
Q34070244 | On-tissue localization of ceramides and other sphingolipids by MALDI mass spectrometry imaging. |
Q94465770 | Pre-clinical Mouse Models of Neurodegenerative Lysosomal Storage Diseases |
Q50079745 | Sphingolipid pathway enzymes modulate cell fate and immune responses |
Q55248134 | Structural basis for the activation of acid ceramidase. |
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