Results 61 to 70 of about 9,480 (220)

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

Adiponectin improves coronary no-reflow injury by protecting the endothelium in rats with type 2 diabetes mellitus. [PDF]

, 2017
To determine the effect of adiponectin (APN) on the coronary no-reflow (NR) injury in rats with Type 2 diabetes mellitus (T2DM), 80 male Sprague-Dawley rats were fed with a high-sugar-high-fat diet to build a T2DM model.
Han, Xue, Liu, Huirong, Liu, Xin, Lv, Tingting, Ma, Lu, Ma, Xin-Liang, Sun, Qi, Wang, Ke, Wang, Wen, Wu, Ye, Xu, Wenli, Zhang, Suli   +11 more
core   +2 more sources

Membrane topology of yeast alkaline ceramidase YPC1 [PDF]

Biochemical Journal, 2013
Ypc1p (yeast phyto-ceramidase 1) and Ydc1p (yeast dihydroceramidase 1) are alkaline ceramide hydrolases that reside in the ER (endoplasmic reticulum). Ypc1p can catalyse the reverse reaction, i.e. the condensation of non-esterified fatty acids with phytosphingosine or dihydrosphingosine and overexpression of YPC1 or YDC1 can provide enough ceramide ...
Ramachandra, Nagaraju, Conzelmann, Andreas   +1 more
openaire   +2 more sources

ORMDL Proteins Turnover via Proteasome and Autophagy Is Cell‐Type Dependent and Tied to Ceramide Homeostasis

The FASEB Journal, Volume 40, Issue 6, 31 March 2026.
ORMDL proteins in the endoplasmic reticulum (ER) negatively regulate serine palmitoyltransferase (SPT) activity in response to the rate of de novo sphingolipid synthesis. Disruption of ceramide homeostasis by myriocin or fumonisin B1 (FB1) appears to destabilize the ORMDL‐SPT complex, leading to ORMDL degradation as a feedback mechanism.
Michal Mrkacek, Magda Tumova, Alex Puskasu, Pavol Utekal, Marek Vrbacky, Ladislav Kuchar, Petr Draber, Viktor Bugajev   +7 more
wiley   +1 more source

Alkaline Ceramidase 1 Protects Mice from Premature Hair Loss by Maintaining the Homeostasis of Hair Follicle Stem Cells

Stem Cell Reports, 2017
Summary: Ceramides and their metabolites are important for the homeostasis of the epidermis, but much remains unknown about the roles of specific pathways of ceramide metabolism in skin biology.
Chih-Li Lin, Ruijuan Xu, Jae Kyo Yi, Fang Li, Jiang Chen, Evan C. Jones, Jordan B. Slutsky, Liqun Huang, Basil Rigas, Jian Cao, Xiaoming Zhong, Ashley J. Snider, Lina M. Obeid, Yusuf A. Hannun, Cungui Mao   +14 more
doaj   +1 more source

Acid ceramidase of macrophages traps herpes simplex virus in multivesicular bodies and protects from severe disease

Nature Communications, 2020
Macrophages are critical in limiting replication of herpes simplex virus type 1 (HSV-1). Here the authors show how acid ceramidase and its enzymatic product sphingosine enable multivesicular bodies to function as an anti-viral mechanism.
Judith Lang, Patrick Bohn, Hilal Bhat, Holger Jastrow, Bernd Walkenfort, Feyza Cansiz, Julian Fink, Michael Bauer, Dominik Olszewski, Ana Ramos-Nascimento, Vikas Duhan, Sarah-Kim Friedrich, Katrin Anne Becker, Adalbert Krawczyk, Michael J. Edwards, Andreas Burchert, Magdalena Huber, Justa Friebus-Kardash, Joachim R. Göthert, Cornelia Hardt, Hans Christian Probst, Fabian Schumacher, Karl Köhrer, Burkhard Kleuser, Eduard B. Babiychuk, Beate Sodeik, Jürgen Seibel, Urs F. Greber, Philipp A. Lang, Erich Gulbins, Karl S. Lang   +30 more
doaj   +1 more source

The clinical spectrum of SMA‐PME and in vitro normalization of its cellular ceramide profile

Annals of Clinical and Translational Neurology, 2022
Objective The objectives of this study were to define the clinical and biochemical spectrum of spinal muscular atrophy with progressive myoclonic epilepsy (SMA‐PME) and to determine if aberrant cellular ceramide accumulation could be normalized by enzyme
Michelle M. Lee, Graeme S. V. McDowell, Darryl C. De Vivo, Daniel Friedman, Samuel F. Berkovic, Maria Spanou, Argirios Dinopoulos, Katheryn Grand, Pedro A. Sanchez‐Lara, Michelle Allen‐Sharpley, Jodi Warman‐Chardon, Alexander Solyom, Thierry Levade, Edward H. Schuchman, Steffany A. L. Bennett, David A. Dyment, Toni S. Pearson   +16 more
doaj   +1 more source

Chemical approaches to sphingolipid research [PDF]

, 2015
Podeu consultar el llibre complet a: http://hdl.handle.net/2445 ...
Abad Saiz, José Luis, Casas Brugulat, Josefina, Delgado Cirilo, Antonio, Fabriàs Domingo, Gemma   +3 more
core  

Characterization of yeast mutants lacking alkaline ceramidases YPC1 and YDC1 [PDF]

, 2014
Humans and yeast possess alkaline ceramidases located in the early secretory pathway. Single deletions of the highly homologous yeast alkaline ceramidases YPC1 and YDC1 have very little genetic interactions or phenotypes.
Cerantola, Vanessa, Conzelmann, Andreas, Ejsing, Christer S., Knudsen, Jens, Mallela, Shamroop K., Sonderegger, Mélanie, Vazquez, Hector M., Voynova, Natalia S.   +7 more
core   +1 more source

Improved synthesis of a fluorogenic ceramidase substrate [PDF]

Bioorganic & Medicinal Chemistry, 2010
Substantial interest has focused on the roles of sphingolipid metabolizing enzymes in a variety of hyperproliferative and inflammatory diseases. A key family of enzymes involved in these pathologies is the ceramidases. Ceramidases cleave the pro-apoptotic lipid ceramide into a long-chain fatty acid and sphingosine, which can then be further metabolized
Xia, Zuping, Draper, Jeremiah M, Smith, Charles D   +2 more
openaire   +3 more sources