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Hematopoietic stem cell transplantation leads to biochemical and functional correction in two mouse models of acid ceramidase deficiency. [PDF]
Rybova J +6 more
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Acid ceramidase expression reduces IFNγ secretion by mouse CD4<sup>+</sup> T cells and is crucial for maintaining B-cell numbers in mice. [PDF]
Mandasari P +8 more
europepmc +1 more source
Inhibition of acid ceramidase as a therapeutic strategy for Niemann-Pick C disease
Haynes I +4 more
europepmc +1 more source
Sphingolipids and acid ceramidase as therapeutic targets in cancer therapy [PDF]
Background: Sphingolipids have been shown to play a key part in cancer cell growth and death and have increasingly become the subject of novel anti-cancer therapies.
Dale Vimalachandran +2 more
exaly +4 more sources
New insights on the use of desipramine as an inhibitor for acid ceramidase [PDF]
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)
James Norris +2 more
exaly +5 more sources
Acid ceramidase and human disease
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).
Edward H Schuchman, Jae Ho Park
exaly +3 more sources
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The molecular medicine of acid ceramidase
Biological Chemistry, 2015Abstract 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 two rare inherited disorders, Farber lipogranulomatosis (Farber disease ...
Michael, Frohbergh +2 more
openaire +2 more sources
Properties of acid ceramidase from human spleen
Lipids and Lipid Metabolism, 1989We have characterised ceramidase activity in extracts of human spleen from control subjects and from patients with Gaucher disease. In Triton X-100 extracts of control spleens, a broad pH optimum of pH 3.5-5.0 was found; no ceramidase activity was detectable at neutral or alkaline pH.
Joseph M Tager, A W Schram, Hugo W Moser
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