TMET-29. TARGETING IDH1-MUTATED OLIGODENDROGLIOMA WITH ACID CERAMIDASE INHIBITORS [PDF]
Neuro OncolCurrently, an estimated 14,950 people lives with oligodendroglioma in the United States. Oligodendroglioma is genetically defined as a tumor harboring isocitrate dehydrogenase 1 or 2 mutations (IDH1mut/IDH2mut).
Helena Muley Vilamu +3 more
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Acid ceramidase targeting pyruvate kinase affected trypsinogen activation in acute pancreatitis [PDF]
Mol Med, 2022 Acute pancreatitis is the sudden inflammation of the pancreas. Severe cases of acute pancreatitis are potentially fatal and have no specific treatment available. Premature trypsinogen activation could initiate acute pancreatitis.
Juan Xiao +4 more
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Acid ceramidase ASAH1 is a key regulator of epidermal ceramide levels and composition [PDF]
J Biol ChemMaintenance of appropriate ceramide levels and composition in the stratum corneum of the epidermis is essential for skin barrier function. Although ceramide homeostasis is regulated by both synthesis and degradation, the extent of ceramide degradation in
Wakana Nobumoto +5 more
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Skin inflammation and impaired adipogenesis in a mouse model of acid ceramidase deficiency [PDF]
J Inherit Metab Dis, 2022 Acid ceramidase catalyzes the degradation of ceramide into sphingosine and a free fatty acid. Acid ceramidase deficiency results in lipid accumulation in many tissues and leads to the development of Farber disease (FD).
J. Rybova +4 more
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Regulation of exosome release by lysosomal acid ceramidase in coronary arterial endothelial cells: Role of TRPML1 channel [PDF]
Curr Top Membr, 2022 Lysosomal acid ceramidase (AC) has been reported to determine multivesicular body (MVB) fate and exosome secretion in different mammalian cells including coronary arterial endothelial cells (CAECs). However, this AC-mediated regulation of exosome release
Guangbi Li +5 more
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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
doaj +3 more sources
Inhibition of acid ceramidase as a therapeutic strategy for Niemann-Pick C disease
bioRxivLysosomal storage disorders (LSDs) are a group of individually rare diseases that as a group constitute the most common forms of childhood neurodegeneration. These life limiting and life shortening diseases have the largest economic burden per patient of
I. Haynes +4 more
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Acid ceramidase controls apoptosis and increases autophagy in human melanoma cells treated with doxorubicin [PDF]
Sci Rep, 2021 Acid ceramidase (AC) is a lysosomal hydrolase encoded by the ASAH1 gene, which cleaves ceramides into sphingosine and fatty acid. AC is expressed at high levels in most human melanoma cell lines and may confer resistance against chemotherapeutic agents ...
Michele Lai +8 more
semanticscholar +2 more sources
New insights on the use of desipramine as an inhibitor for acid ceramidase [PDF]
FEBS Letters, 2006 Treatment of different cancer cell lines with desipramine induced a time‐ and dose‐dependent downregulation of acid ceramidase. Desipramine's effect on acid ceramidase appeared specific for amphiphilic agents (desipramine, chlorpromazine, and chloroquine) but not other lysomotropic agents such as ammonium chloride and bafilomycin A1, and was not ...
Saeed Elojeimy +2 more
exaly +3 more sources
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