scholarly journals Autoproteolytic Cleavage and Activation of Human Acid Ceramidase

2008 ◽  
Vol 283 (17) ◽  
pp. 11253-11259 ◽  
Author(s):  
Nataly Shtraizent ◽  
Efrat Eliyahu ◽  
Jae-Ho Park ◽  
Xingxuan He ◽  
Ruth Shalgi ◽  
...  
Keyword(s):  
Acid Ceramidase ◽  
Human Acid ◽  
Autoproteolytic Cleavage

Human acid ceramidase is overexpressed but not mutated in prostate cancer

2000 ◽  
Vol 29 (2) ◽  
pp. 137-146 ◽  
Author(s):  
Ratnam S. Seelan ◽  
Chiping Qian ◽  
Akira Yokomizo ◽  
David G. Bostwick ◽  
David I. Smith ◽  
...  
Keyword(s):  
Prostate Cancer ◽  
Acid Ceramidase ◽  
Human Acid

Pharmacokinetics and tissue distribution of RVT-801, a recombinant human acid ceramidase, at efficacious doses in a murine model of Farber disease

2018 ◽  
Vol 123 (2) ◽  
pp. S50
Author(s):  
Eric Gaukel ◽  
Christine Coquery ◽  
Brante Sampey ◽  
Edward Schuchman ◽  
Stephen A. Wring
Keyword(s):  
Tissue Distribution ◽  
Murine Model ◽  
Acid Ceramidase ◽  
Farber Disease ◽  
Human Acid

Expression of human acid ceramidase in insect cells. Analysis of processing, glycosylation and disulfide bond pattern.

2007 ◽  
Vol 2007 (Spring) ◽  
Author(s):  
Heike Schulze ◽  
Oliver Bartelsen ◽  
Ute Schepers ◽  
Konrad Sandhoff
Keyword(s):  
Disulfide Bond ◽  
Insect Cells ◽  
Acid Ceramidase ◽  
Human Acid

Prediction of the human equivalent dose (HED) of RVT-801, a recombinant human acid ceramidase, for the treatment of Farber disease

2018 ◽  
Vol 123 (2) ◽  
pp. S50
Author(s):  
Eric Gaukel ◽  
Christine Coquery ◽  
Brante Sampey ◽  
Edward Schuchman ◽  
Stephen A. Wring
Keyword(s):  
Equivalent Dose ◽  
Acid Ceramidase ◽  
Farber Disease ◽  
Human Acid

scholarly journals Purification, characterization, and biosynthesis of human acid ceramidase

1995 ◽  
Vol 270 (19) ◽  
pp. 11098-11102 ◽  
Author(s):  
K Bernardo ◽  
R Hurwitz ◽  
T Zenk ◽  
RJ Desnick ◽  
K Ferlinz ◽  
...  
Keyword(s):  
Acid Ceramidase ◽  
Human Acid

scholarly journals Human Acid Ceramidase

2001 ◽  
Vol 276 (38) ◽  
pp. 35352-35360 ◽  
Author(s):  
Klaus Ferlinz ◽  
Guido Kopal ◽  
Katussevani Bernardo ◽  
Thomas Linke ◽  
Julia Bär ◽  
...  
Keyword(s):  
Acid Ceramidase ◽  
Human Acid

scholarly journals Overexpression of Acid Ceramidase Protects from Tumor Necrosis Factor–Induced Cell Death

2000 ◽  
Vol 192 (5) ◽  
pp. 601-612 ◽  
Author(s):  
Astrid Strelow ◽  
Katussevani Bernardo ◽  
Sabine Adam-Klages ◽  
Thomas Linke ◽  
Konrad Sandhoff ◽  
...  
Keyword(s):  
Cell Death ◽  
Tumor Necrosis Factor ◽  
Tumor Necrosis ◽  
Acid Sphingomyelinase ◽  
Acid Ceramidase ◽  
L929 Cells ◽  
Human Acid ◽  
Sphingosine 1 Phosphate ◽  
Dependent Cell ◽  
Necrosis Factor

Tumor necrosis factor (TNF) signals cell death and simultaneously induces generation of ceramide. To evaluate the contribution of ceramide to TNF-dependent cell death, we generated clones of the TNF-sensitive cell line L929 that constitutively overexpress human acid ceramidase (AC). Ceramidase, in concert with sphingosine kinase, metabolizes ceramide to sphingosine-1-phosphate (SPP), an inducer of proliferation. In response to TNF, parental L929 cells display a significant increase in intracellular ceramide correlated with an “atypical apoptosis” characterized by membrane blebbing, DNA fragmentation and degradation of poly(ADP-ribose) polymerase despite a lack of caspase activity. These features are strongly reduced or absent in AC-overexpressing cells. Pharmacological suppression of AC with N-oleoylethanolamine restored the accumulation of intracellular ceramide as well as the sensitivity of the transfectants to TNF, implying that an enhanced metabolization of intracellular ceramide by AC shifts the balance between intracellular ceramide and SPP levels towards cell survival. Correspondingly, inhibition of ceramide production by acid sphingomyelinase also increased survival of TNF-treated L929 cells.

The Human Acid Ceramidase Gene (ASAH): Structure, Chromosomal Location, Mutation Analysis, and Expression

Genomics ◽  
1999 ◽  
Vol 62 (2) ◽  
pp. 223-231 ◽  
Author(s):  
Chi-Ming Li ◽  
Jae-Ho Park ◽  
Xingxuan He ◽  
Brynn Levy ◽  
Fei Chen ◽  
...  
Keyword(s):  
Mutation Analysis ◽  
Chromosomal Location ◽  
Acid Ceramidase ◽  
Human Acid
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