Results 31 to 40 of about 2,521,035 (177)

Interferon regulatory factor 8 regulates expression of acid ceramidase and infection susceptibility in cystic fibrosis [PDF]

, 2021
\ua9 2021 THE AUTHORS. Published by Elsevier Inc on behalf of American Society for Biochemistry and Molecular Biology. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).Most patients with ...
Keitsch S, Becker KA, Gardner AI, Verhaegh R, Soddemann M, Edwards MJ, Haq IJ, Liu Y, Wilker B, Gulbins E, Brodlie M, Kamler M, Wu Y   +12 more
core   +4 more sources

Recombinant Acid Ceramidase Reduces Inflammation and Infection in Cystic Fibrosis. [PDF]

Am J Respir Crit Care Med, 2020
Rationale: In cystic fibrosis the major cause of morbidity and mortality is lung disease characterized by inflammation and infection. The influence of sphingolipid metabolism is poorly understood with a lack of studies using human airway model systems ...
Gardner AI, Haq IJ, Simpson AJ, Becker KA, Gallagher J, Saint-Criq V, Verdon B, Mavin E, Trigg A, Gray MA, Koulman A, McDonnell MJ, Fisher AJ, Kramer EL, Clancy JP, Ward C, Schuchman EH, Gulbins E, Brodlie M.   +18 more
europepmc   +3 more sources

Autoproteolytic Cleavage and Activation of Human Acid Ceramidase [PDF]

Journal of Biological Chemistry, 2008
Herein we report the mechanism of human acid ceramidase (AC; N-acylsphingosine deacylase) cleavage and activation. A highly purified, recombinant human AC precursor underwent self-cleavage into alpha and beta subunits, similar to other members of the N-terminal nucleophile hydrolase superfamily.
Nataly, Shtraizent, Efrat, Eliyahu, Jae-Ho, Park, Xingxuan, He, Ruth, Shalgi, Edward H, Schuchman   +5 more
openaire   +2 more sources

New fluorogenic probes for neutral and alkaline ceramidases

Journal of Lipid Research, 2019
New fluorogenic ceramidase substrates derived from the N-acyl modification of our previously reported probes (RBM14) are reported. While none of the new probes were superior to the known RBM14C12 as acid ceramidase substrates, the corresponding nervonic ...
Mireia Casasampere, Núria Bielsa, Daniel Riba, Laura Bassas, Ruijuan Xu, Cungui Mao, Gemma Fabriàs, José-Luis Abad, Antonio Delgado, Josefina Casas   +9 more
doaj   +1 more source

Purification, Characterization, and Biosynthesis of Human Acid Ceramidase [PDF]

Journal of Biological Chemistry, 1995
Acid ceramidase (N-acylsphingosine deacylase, EC 3.5.1.23) is the lysosomal enzyme catalyzing the hydrolysis of ceramide to sphingosine and free fatty acid. Its inherited deficiency causes ceramide accumulation in Farber's disease. The enzyme was purified to apparent homogeneity from human urine by sequential chromatography on octyl-Sepharose ...
K, Bernardo, R, Hurwitz, T, Zenk, R J, Desnick, K, Ferlinz, E H, Schuchman, K, Sandhoff   +6 more
openaire   +2 more sources

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, Alexandra Berger, Caren Furlonger, Joshua M. Moreau, Makoto Yoshimitsu, Jessa Trentadue, Bryan C.Y. Au, Christopher J. Paige, Jeffrey A. Medin   +8 more
doaj   +3 more sources

Potent Inhibition of Acid Ceramidase by Novel B-13 Analogues [PDF]

, 2011
The lipid-signalling molecule ceramide is known to induce apoptosis in a variety of cell types. Inhibition of the lysosomal acid ceramidase can increase cellular ceramide levels and thus induce apoptosis.
Jan Jasper Klein, Christoph Arenz, Denny Proksch   +2 more
core   +1 more source

Molecular Mechanism of Inhibition of Acid Ceramidase by Carmofur [PDF]

Journal of Medicinal Chemistry, 2018
Human acid ceramidase (AC) is a lysosomal cysteine amidase, which has received a great deal of interest in recent years as a potential target for the development of new therapeutics against melanoma and glioblastoma tumors. Despite the strong interest in obtaining structural information, only the structures of the apo-AC enzyme in its zymogen and ...
Alexey Dementiev, Andrzej Joachimiak, Ha Nguyen, Alexei Gorelik, Katalin Illes, Saman Shabani, Michael Gelsomino, Eun-Young Erin Ahn, Bhushan Nagar, Ninh Doan   +9 more
openaire   +2 more sources

Stinging Nettle (Urtica dioica L.) Attenuates FFA Induced Ceramide Accumulation in 3T3-L1 Adipocytes in an Adiponectin Dependent Manner.

PLoS ONE, 2016
ObjectiveExcess dietary lipids result in the accumulation of lipid metabolites including ceramides that can attenuate insulin signaling. There is evidence that a botanical extract of Urtica dioica L.
Diana N Obanda, Peng Zhao, Allison J Richard, David Ribnicky, William T Cefalu, Jacqueline M Stephens   +5 more
doaj   +1 more source

Adiponectin receptor agonist ameliorates cardiac lipotoxicity via enhancing ceramide metabolism in type 2 diabetic mice

Cell Death and Disease, 2022
Accumulation of lipids and their metabolites induces lipotoxicity in diabetic cardiomyopathy. Lowering ceramide concentration could reduce the impact of metabolic damage to target organs. Adiponectin improves lipotoxicity through its receptors (AdiopRs),
Yaeni Kim, Ji Hee Lim, Eun Nim Kim, Yu Ah Hong, Hun-Jun Park, Sungjin Chung, Bum Soon Choi, Yong-Soo Kim, Ji Yong Park, Hye Won Kim, Cheol Whee Park   +10 more
doaj   +1 more source