Results 11 to 20 of about 8,571 (202)
Structural basis for the activation of acid ceramidase [PDF]
Nature Communications, 2018 Acid ceramidase (aCDase) hydrolyzes lysosomal membrane ceramide into sphingosine and its dysfunction leads to a variety of disease phenotypes. Here, the authors present structures of aCDase in its proenzyme and autocleaved forms, which provides insight ...
Ahmad Gebai +4 more
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Adiponectin overexpression improves metabolic abnormalities caused by acid ceramidase deficiency but does not prolong lifespan in a mouse model of Farber Disease [PDF]
Molecular Genetics and Metabolism ReportsFarber Disease is a debilitating and lethal childhood disease of ceramide accumulation caused by acid ceramidase deficiency. The potent induction of a ligand-gated neutral ceramidase activity promoted by adiponectin may provide sufficient lowering of ...
Marie K. Norris +8 more
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Acid ceramidase deficiency: Farber disease and SMA-PME [PDF]
Orphanet Journal of Rare Diseases, 2018 Acid ceramidase (ACDase) deficiency is a spectrum of disorders that includes a rare lysosomal storage disorder called Farber disease (FD) and a rare epileptic disorder called spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME).
Fabian P. S. Yu +3 more
doaj +3 more sources
Acid Ceramidase Rescues Cystic Fibrosis Mice from Pulmonary Infections. [PDF]
Infect Immun, 2021 Previous studies have shown that sphingosine kills a variety of pathogenic bacteria, including Pseudomonas aeruginosa and Staphylococcus aureus . Sphingosine concentrations are decreased in airway epithelial cells of cystic fibrosis (CF) mice, and this defect has been linked to the infection ...
Becker KA +10 more
europepmc +4 more sources
Targeting Acid Ceramidase Inhibits Glioblastoma Cell Migration through Decreased AKT Signaling [PDF]
Cells, 2022 Glioblastoma (GBM) remains one of the most aggressive cancers, partially due to its ability to migrate into the surrounding brain. The sphingolipid balance, or the balance between ceramides and sphingosine-1-phosphate, contributes to the ability of GBM ...
Cyntanna C. Hawkins +12 more
doaj +2 more sources
Acid ceramidase and human disease
Biochimica et Biophysica Acta (BBA) - Biomembranes, 2006 Acid ceramidase (N-acylsphingosine deacylase, EC 3.5.1.23; AC) is the lipid hydrolase responsible for the degradation of ceramide into sphingosine and free fatty acids within lysosomes. The enzymatic activity was first identified over four decades ago, and is deficient in the inherited lipid storage disorder, Farber Lipogranulomatosis (Farber disease).
Park, Jae-Ho, Schuchman, Edward H.
openaire +4 more sources
Targeting acid ceramidase ameliorates fibrosis in mouse models of non-alcoholic steatohepatitis [PDF]
Frontiers in Medicine, 2022 Non-alcoholic fatty liver disease (NAFLD) is a common cause of liver disease worldwide, and is characterized by the accumulation of fat in the liver.
Amy Yu +11 more
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Effects of flavonoids on sphingolipid turnover in the toxin-damaged liver and liver cells [PDF]
Lipids in Health and Disease, 2008 Background The ceramide generation is an early event in the apoptotic response to numerous stimuli including the oxidative stress and ceramide analogs mimic the stress effect and induce apoptosis.
Shakhova Elena G, Babenko Nataliya A
doaj +4 more sources
Pharmaceutics, 2018 Glioblastoma is the most common, malignant primary tumor of the central nervous system. The average prognosis for life expectancy after diagnosis, with the triad of surgery, chemotherapy, and radiation therapy, is less than 1.5 years.
Ha S. Nguyen +3 more
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Markedly perturbed hematopoiesis in acid ceramidase deficient mice
Haematologica, 2015 Acid ceramidase (ACDase) is ubiquitous and catalyzes the degradation of ceramide. ACDase and ceramides have been implicated in many disorders, including cancer, obesity, diabetes, inflammation, and neurodegenerative diseases.[1][1]–[3][2] Deficiencies in ACDase activity lead to Farber disease ...
Shaalee Dworski +8 more
doaj +3 more sources