نتایج جستجو برای: acid ceramidase
تعداد نتایج: 747667 فیلتر نتایج به سال:
Abstract The presence of the IDH mutation in gliomas is a major classifier brain tumor subtypes and has several important implications for cancer growth. Our recent work uncovered that IDH-mutant tumors are susceptible to increased apoptosis via alterations sphingolipid pathway due their excess production pro-apoptotic ceramides over pro-proliferative sphingosine 1-phosphate (S1P). To end, we p...
Abbreviations: TEWL: Transepidermal Water Loss; AD: Atopic Dermatitis; SC: Stratum Corneum; HC: Healthy Control; PiCl: Picryl Chloride; MA: House Dust Mite Antigens; SM: Sphingomyelin; GCer: Glucosylceramide; BGCase: Beta-Glucocerebrosidase; SMase: Acid Sphingomyelinase; CBE: Conduritol B Epoxide; DSP: Despiramine Hydrochloride; PDMP: 1-Phenyl-2-Decanoylamino-3-Morpholino-1Propanol; SPT: Serine...
Analysis of lipid storage in postmortem brains of patients with amaurotic idiocy led to the recognition of five lysosomal ganglioside storage diseases and identification of their inherited metabolic blocks. Purification of lysosomal acid sphingomyelinase and ceramidase and analysis of their gene structures were the prerequisites for the clarification of Niemann-Pick and Farber disease. For lipi...
During the last decade, sphingolipid deregulation, namely the balance between the pro-apoptotic molecule ceramide and the anti-apoptotic sphingolipid sphingosine-1-phosphate, has emerged as an important factor in cancer pathology and resistance to therapy. Thus, our research has been focused on developing drugs that are able to restore normal sphingolipid balance, precisely through increasing t...
Glioblastoma multiforme (GBM) is the most common primary, intracranial malignancy of the central nervous system. The standard treatment protocol, which involves surgical resection, and concurrent radiation with adjuvant temozolomide (TMZ), still imparts a grim prognosis. Ultimately, all GBMs exhibit recurrence or progression, developing resistance to standard treatment. This study demonstrates ...
BACKGROUND/AIMS Leukodystrophies due to abnormal production of myelin cause extensive morbidity in early life; their genetic background is still largely unknown. We aimed at reaching a molecular diagnosis in Ashkenazi-Jewish patients who suffered from developmental regression at 6-13 months, leukodystrophy and peripheral neuropathy. METHODS Exome analysis, determination of alkaline ceramidase...
A series of structurally related 2,4-dioxopyrimidine-1-carboxamide derivatives as highly potent inhibitors against acid ceramidase were subjected to hologram quantitative structure-activity relationship (HQSAR) analysis. A training set containing 24 compounds served to establish the HQSAR model. The best HQSAR model was generated using atoms, bond, connectivity, donor and acceptor as fragment d...
Neutral ceramidase activity has previously been identified in the intestinal mucosa and gut lumen and postulated to be important in the digestion of sphingolipids. It is found throughout the intestine but has never been fully characterized. We have purified rat intestinal neutral ceramidase from an eluate obtained by perfusing the intestinal lumen with 0.9% NaCl and 3 mM sodium taurodeoxycholat...
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