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Properties of acid ceramidase from human spleen
Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1989 We 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.B J, Al, C W, Tiffany, D S, Gomes de Mesquita, H W, Moser, J M, Tager, A W, Schram +5 moreopenaire +2 more sourcesDevelopment of an acid ceramidase activity-based probe
Chemical Communications, 2015 Acid ceramidase is responsible for the ultimate step in the catabolism of (glyco)sphingolipids by hydrolysis of ceramide into sphingosine and free fatty acid.Ouairy, C.M.J., Ferraz, M.J., Boot, R.G., Baggelaar, M.P., Stelt, M. van der, Appelman, M., Marel, G.A. van der, Florea, B.I., Aerts, J.M.F.G., Overkleeft, H.S. +9 moreopenaire +3 more sourcesAcid Ceramidase: A Novel Therapeutic Target in Cancer
Current Topics in Medicinal Chemistry, 2019 Sphingolipids are important constituents of the eukaryotic cell membrane which govern various signaling pathways related to different aspects of cell survival. Ceramide and Sphingosine are interconvertible sphingolipid metabolites, out of which Ceramide is pro-apoptotic and sphingosine is anti-apoptotic in nature.Yadu, Vijayan, Manendra Babu, Lankadasari, Kuzhuvelil B, Harikumar +2 moreopenaire +2 more sourcesHuman Acid Ceramidase Gene: Novel Mutations in Farber Disease
Molecular Genetics and Metabolism, 2000 Farber disease is an autosomal recessive disorder caused by lysosomal acid ceramidase (AC) deficiency. It commonly manifests during the first few months after birth with a unique triad of painful and progressive deformed joints, subcutaneous nodules, and progressive hoarseness. In order to understand the molecular mechanism(s) of pathogenesis of Farber Z, Zhang, A K, Mandal, A, Mital, N, Popescu, D, Zimonjic, A, Moser, H, Moser, A B, Mukherjee +7 moreopenaire +2 more sourcesAcid ceramidase inhibition: a novel target for cancer therapy
Frontiers in Bioscience, 2008 During the last decade, sphingolipid deregulation, namely the balance between the pro-apoptotic molecule ceramide and the anti-apoptotic sphingolipid sphingosine-1-phosphate, has emerged as an important factor in cancer pathology and resistance to therapy.Liu, X, Elojeimy, S, Turner, L S, Mahdy, A E M, Zeidan, Y H, Bielawska, A, Bielawski, J, Dong, J Y, El-Zawahry, A M, Guo, G W, Hannun, Y A, Holman, D H, Rubinchik, S, Szulc, Z, Keane, T E, Tavassoli, M, Norris, J S +16 moreopenaire +3 more sourcesKinetic Characteristics of Acidic and Alkaline Ceramidase in Human Epidermis
Skin Pharmacology and Physiology, 2007 It has recently become evident that at least five ceramidase (CDase) isoforms are present in human epidermis, and that specifically acidic CDase (aCDase) and alkaline CDase (alkCDase) activities increase during keratinocyte differentiation, and thus might play a pivotal role(s) in permeability barrier function.Houben, E., Uchida, Y., Nieuwenhuizen, W.F., Paepe, K. de, Vanhaecke, T., Holleran, W.M., Rogiers, V. +6 moreopenaire +4 more sourcesTargeting IDH1-Mutated Oligodendroglioma with Acid Ceramidase Inhibitors
AbstractOligodendroglioma is genetically defined as a tumor harboring isocitrate dehydrogenase 1 or 2 mutations (IDH1mut/IDH2mut) and 1p/19q co-deletions. Previously, we reported that in IDH1mutgliomas, D-2HG, the product of IDH1 mutant enzyme produces an increase in monounsaturated fatty acid levels that are incorporated into ceramides, tilting the ...Helena Muley, Tyrone Dowdy, Faris Zaibaq, George Karadimov, Aiguo Li, Hua Song, Meili Zhang, Wei Zhang, Zalman Wong, Laurence Zhang, Adrian Lita, Mioara Larion +11 moreopenaire +2 more sourcesTargeting Acid Ceramidase in Acute Myeloid Leukemia
2019 Acute myeloid leukemia (AML) is a hematologic malignancy characterized by expansion of myeloid blasts. These immature cells overpopulate the blood and bone marrow leading to anemia, neutropenia and thrombocytopenia associated with increased risk of infection. AML progresses rapidly and many patients relapse after treatment.openaire +1 more sourceAAV-mediated gene therapy of acid ceramidase deficiency
2022 Acid ceramidase deficiency is an orphan disorder caused by mutations in the ASAH1 genepresenting with a spectrum of clinical manifestations that leads to death in most cases duringearly infancy (Farber disease, FD) or at teenage (spinal muscular atrophy with progressivemyoclonic epilepsy, SMA-PME).Denard, Jérôme, Marinello, Martina, Latournerie, Virginie, Bonnin, Delphine, Derome, Marion, Martin, Samia, Medin, Jeffrey, Buj-Bello, Ana +7 moreopenaire +1 more sourceAcid Ceramidase Deficiency
2009 Hubert Scharnagl, Winfried März, Markus Böhm, Thomas A. Luger, Federico Fracassi, Alessia Diana, Thomas Frieling, Susanna Mac, Dan Donoghue, Susanne Kohl, Gema Ariceta, Daniel Batlle, Gema Ariceta, Daniel Batlle, Daniel Batlle, Ehteshamudin Syed, Jacob I. Sznajder, Christos C. Zouboulis, Markus Pfister, Deepak Kamat, Alexander K. C. Leung, Thomas Schwarz, Jorge Frank, Sven Schinner, Stefan R. Bornstein, Meinhard Schiller, Dorothée Nashan, Cord Sunderkötter, Thomas Schwarz, Mark Berneburg, Nigel G. Laing, Alan H. Beggs, Hans H. Goebel, Du Toit Loots, Amrik Sahota, Jay A. Tischfield, H. Anne Simmonds, Wolfgang Dietmaier, Arndt Hartmann, Michael S. Hershfield, Georges Berghe, Jaak Jaeken, Manish Suneja, Christie P. Thomas, Manish Suneja, Christie P. Thomas, Holger S. Willenberg, Stefan R. Bornstein, Holger S. Willenberg, James M. Powers, James M. Powers, Herbert L. Bonkovsky, Manish Thapar, Sishir Mathur, Lee A. Denson, André B. P. Kuilenburg, Albert H. Gennip, Wim Hul, Filip Vanhoenacker, Markus Böhm, Thomas A. Luger, Karel Frasch, Markus Jäger, Nilufar Mohebbi, Carsten A. Wagner, Malin Lindstedt, Carl Borrebaeck, Michala Bezdekova, Svetlana Brychtova, Zdenek Kolar, Tomas Brychta, Renata Kucerova, Martina Bienova, Tobias W. Fischer, Ralf Paus, Birgit Haack, Stefan Kins, Konrad Beyreuther, André B. P. Kuilenburg, Albert H. Gennip, Sehsuvar Ertürk, Sim Kutlay, Michael Emberger, Helmut Hintner, Thomas Hawranek, Ruth Danzeisen, Albert C. Ludolph, Michelle M. Estrella, Mohamed G. Atta, Kumarasamy Thangaraj, Singh Rajender, John-John B. Schnog, Victor E. A. Gerdes, Karina Yazdanbakhsh, Andromachi Scaradavou, Norbert Gattermann, Michael L. H. Ma, Patrick T. S. Ma, Alexander K. C. Leung, Alessandra Baumer, Wolfgang Schillinger, Gerd Hasenfuss, Matthias Wenning, Murat Bas, Georg Kojda, Randolf Brehler, Engin Sezer, Atilla Şenaylı, Michael Stürzl, Peter Klein, Christian Zietz, Steven Mumm, Guido Adler, Kirsty Kiezebrink, Alan P. Knutsen, Cord Sunderkötter, Henrik Larsson, Essi Viding, Hugo Ten Cate, James K. Stoller, David A. Lomas, Daniela Eser, Rainer Rupprecht, Matthias Siepe, Friedhelm Beyersdorf, Michael L. H. Ma, Patrick T. S. Ma, Alexander K. C. Leung, Volker Liebe, Jens J. Kaden, Raffi Bekeredjian, Norbert Frey, Pavel Fencl, Lucia K. Ma, Patrick T. S. Ma, Alexander K. C. Leung, Omar A. Ibrahimi, Moosa Mohammadi, Michael M. Hoffmann, Winfried März, Sebastian Wesselborg, Björn Stork, Sylvia Stöckler-Ipsiroglu, Avi Fischer, Davendra Mehta, Patrick T. S. Ma, Michael L. H. Ma, Alexander K. C. Leung, Jens Seiler, Martin Rotter, Alexander Leaf, Srijita Sen-Chowdhry, Deirdre Ward, William J. McKenna, Jens Mogensen, Christopher K. Zarins, Roberto Manfredi, Jeno Sebes, Yun Zhao, Darryl C. Zeldin, Pere Ginès, Mónica Guevara, Zhirong Yao, Colin A. Johnson, Damian C. Melles∗, S. Marie, Mark G. Buckley, Gordon Dent, Stephen T. Holgate, Alfried Kohlschütter, Thomas Klockgether, Thomas Klockgether, Thomas Klockgether, Eleanor M. Summerhill, F. Dennis McCool, Rita L. McGill, Mayumi Endo, Rodolfo Paoletti, Andrea Cignarella, Randolf Brehler, M. Cristina Digilio, Bruno Marino, Anna Sarkozy, Bruno Dallapiccola, Kurt C. Roberts-Thomson, Jonathan M. Kalman, Michael L. H. Ma, Patrick T. S. Ma, Alexander K. C. Leung, Andy Wessels, Tim McQuinn, Siobhan D. Ma, Patrick T. S. Ma, Alexander K. C. Leung, Marc Zimmermann, Haran Burri, Robert Dalla Pozza, Klaus-Peter Lesch, Thomas Bourgeron, Nada Kanaan +199 moreopenaire +1 more source
