Results 51 to 60 of about 5,986,972 (170)

The Reverse Activity of Human Acid Ceramidase [PDF]

Journal of Biological Chemistry, 2003
An overexpression system was recently developed to produce and purify recombinant, human acid ceramidase. In addition to ceramide hydrolysis, the purified enzyme was able to catalyze ceramide synthesis using [14C]lauric acid and sphingosine as substrates.
Nozomu, Okino, Xingxuan, He, Shimon, Gatt, Konrad, Sandhoff, Makoto, Ito, Edward H, Schuchman   +5 more
openaire   +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   +2 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

Acid ceramidase promotes nuclear export of PTEN through sphingosine 1-phosphate mediated Akt signaling.

PLoS ONE, 2013
The tumor suppressor PTEN is now understood to regulate cellular processes at the cytoplasmic membrane, where it classically regulates PI3K signaling, as well as in the nucleus where multiple roles in controlling cell cycle and genome stability have been
Thomas H Beckham, Joseph C Cheng, Ping Lu, S Tucker Marrison, James S Norris, Xiang Liu   +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

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

Elevated glucosylsphingosine in Gaucher disease induced pluripotent stem cell neurons deregulates lysosomal compartment through mammalian target of rapamycin complex 1

Stem Cells Translational Medicine, 2021
Gaucher disease (GD) is a lysosomal storage disorder caused by mutations in GBA1, the gene that encodes lysosomal β‐glucocerebrosidase (GCase). Mild mutations in GBA1 cause type 1 non‐neuronopathic GD, whereas severe mutations cause types 2 and 3 ...
Manasa P. Srikanth, Jace W. Jones, Maureen Kane, Ola Awad, Tea Soon Park, Elias T. Zambidis, Ricardo A. Feldman   +6 more
doaj   +1 more source

Inhibition of ceramide accumulation in AdipoR1–/– mice increases photoreceptor survival and improves vision

JCI Insight, 2022
Adiponectin receptor 1 (ADIPOR1) is a lipid and glucose metabolism regulator that possesses intrinsic ceramidase activity. Mutations of the ADIPOR1 gene have been associated with nonsyndromic and syndromic retinitis pigmentosa.
Dominik Lewandowski, Andrzej T. Foik, Roman Smidak, Elliot H. Choi, Jianye Zhang, Thanh Hoang, Aleksander Tworak, Susie Suh, Henri Leinonen, Zhiqian Dong, Antonio F.M. Pinto, Emily Tom, Jennings Luu, Joan Lee, Xiuli Ma, Erhard Bieberich, Seth Blackshaw, Alan Saghatelian, David C. Lyon, Dorota Skowronska-Krawczyk, Marcin Tabaka, Krzysztof Palczewski   +21 more
doaj   +1 more source