Results 61 to 70 of about 7,033 (216)

Targeting the ceramidase ACER3 attenuates cholestasis in mice by mitigating bile acid overload via unsaturated ceramide-mediated LXRβ signaling transduction

Nature Communications
Bile acid overload critically drives the pathogenesis of cholestatic liver injury (CLI). While ceramide metabolism has garnered increasing interest in liver research, the role of ceramides in CLI remains unclear.
Leyi Liao, Ziying Liu, Lei Liu, Can Huang, Yiyi Li, Cungui Mao, R. Xu, Haiqing Liu, Cuiting Liu, Yonghong Peng, Tingying Lei, Hanbiao Liang, Sheng Yu, Jian-ping Qian, Xianqiu Wu, Biao Wang, Yixiong Lin, Jie Zhou, Qingping Li, Chuanjiang Li, Kai Wang   +20 more
semanticscholar   +1 more source

Acid ceramidase improves mitochondrial function and oxidative stress in Niemann-Pick type C disease by repressing STARD1 expression and mitochondrial cholesterol accumulation

Redox Biology, 2021
Niemann-Pick type C (NPC) disease, a lysosomal storage disorder caused by defective NPC1/NPC2 function, results in the accumulation of cholesterol and glycosphingolipids in lysosomes of affected organs, such as liver and brain.
S. Torres, E. Solsona-Vilarrasa, S. Núñez, N. Matías, N. Insausti-Urkia, F. Castro, M. Casasempere, G. Fabriàs, J. Casas, C. Enrich, J. Fernández-Checa, C. Garcia-Ruiz   +11 more
semanticscholar   +1 more source

Deficiency of galactosyl-ceramidase in adult oligodendrocytes worsens disease severity during chronic experimental allergic encephalomyelitis

Molecular Therapy
Galactosyl-ceramidase (GALC) is a ubiquitous lysosomal enzyme crucial for the correct myelination of the mammalian nervous system during early postnatal development.
Natalia Saldivia, Gregory Heller, Diego Zelada, Jason Whitehair, Nikhil Venkat, Ashna Konjeti, Reina Savitzky, Shayla Samano, Daniel Simchuk, R. V. van Breemen, M. Givogri, E. Bongarzone   +11 more
semanticscholar   +1 more source

Acid ceramidase promotes senescent cell survival

Aging, 2021
Cellular senescence is linked to chronic age-related diseases including atherosclerosis, diabetes, and neurodegeneration. Compared to proliferating cells, senescent cells express distinct subsets of proteins. In this study, we used cultured human diploid
R. Munk, C. Anerillas, M. Rossi, D. Tsitsipatis, J. Martindale, Allison B Herman, Jen-Hao Yang, Jackson A. Roberts, V. Varma, Poonam R. Pandey, M. Thambisetty, M. Gorospe, K. Abdelmohsen   +12 more
semanticscholar   +1 more source

CDase is a pan-ceramidase inDrosophila

Molecular Biology of the Cell, 2011
 Ceramidases catalyze the conversion of ceramide to sphingosine. They are acylaminohydrolases that catalyze the deacylation of the amide-linked saturated fatty acid from ceramide to generate sphingosine. They also catalyze the reverse reaction of ceramide biosynthesis using sphingosine and fatty acid.
Yuan, Changqing, Rao, Raghavendra Pralhada, Jesmin, Nahid, Bamba, Takeshi, Nagashima, Kunio, Pascual, Alberto, Preat, Thomas, Fukusaki, Eiichiro, Acharya, Usha, Acharya, Jairaj K.   +9 more
openaire   +3 more sources

Skin inflammation and impaired adipogenesis in a mouse model of acid ceramidase deficiency

Journal of Inherited Metabolic Disease, 2022
Acid ceramidase catalyzes the degradation of ceramide into sphingosine and a free fatty acid. Acid ceramidase deficiency results in lipid accumulation in many tissues and leads to the development of Farber disease (FD).
J. Rybova, L. Kuchař, J. Sikora, W. McKillop, J. Medin   +4 more
semanticscholar   +1 more source

Unraveling A4GALT Mechanism and Its Modulation With Adamantyl‐Galactosylceramide Analogues: Advancing Fabry Disease Therapeutic Strategies

Angewandte Chemie International Edition, EarlyView.
A 310‐helix‐mediated conformational switch promotes a front‐face SNi‐like catalysis by human A4GALT. Mechanism‐guided design identifies AdaGalCer as a selective modulator of globotriaosylceramide (Gb3) biosynthesis, opening a clear route toward new Fabry disease therapeutics.
Nicky de Koster, Òscar Vidal‐Gironès, Rowan de Graaf, Ken Kok, Lindsey T. Lelieveld, Jackelien Hoogervorst, Maria J. Ferraz, José Pablo Rivas‐Fernández, Rob F. Lammers, Herman S. Overkleeft, Johannes M. F. G. Aerts, Rolf G. Boot, Carme Rovira, Marta Artola   +13 more
wiley   +1 more source

The role of ACER2 in intestinal sphingolipid metabolism and gastrointestinal cancers

Frontiers in Immunology
Sphingolipids, particularly sphingosine-1-phosphate (S1P), are bioactive lipids involved in regulating cellular processes such as proliferation, apoptosis, inflammation, and tumor progression.
Binggang Liu, Junfeng Zhou, Biao Jiang, Bing Tang, Ting Liu, Pengcheng Lei   +5 more
doaj   +1 more source

Rare‐Variant Burden across Lysosomal Genes Implicates Sialylation and Ganglioside Metabolism in Parkinson's Disease

Movement Disorders, EarlyView.
Abstract Background Lysosomal dysfunction is central to Parkinson's disease (PD) pathogenesis, with GBA1 representing the strongest established genetic risk factor. Numerous other genes involved in lysosomal sphingolipid, glycosphingolipid, and ceramide metabolism have been proposed as contributors to PD, highlighting the need for genetic analyses ...
Konstantin Senkevich, Sitki Cem Parlar, Cloe Chantereault, Lang Liu, Eric Yu, Uladzislau Rudakou, Jamil Ahmad, Jennifer A. Ruskey, Farnaz Asayesh, Dan Spiegelman, Cheryl Waters, Oury Monchi, Yves Dauvilliers, Nicolas Dupré, Lior Greenbaum, Sharon Hassin‐Baer, Irina Miliukhina, Alla Timofeeva, Anton Emelyanov, Sofya Pchelina, Roy N. Alcalay, Ziv Gan‐Or   +21 more
wiley   +1 more source

Histone deacetylase inhibitors as venetoclax‐sensitising partners in acute myeloid leukaemia: Mechanisms, pharmacology and translational perspectives

British Journal of Pharmacology, EarlyView.
Abstract Venetoclax combined with hypomethylating agents has improved treatment for older or unfit patients with acute myeloid leukaemia (AML), but resistance and relapse remain common. This review analyses the rationale for combining venetoclax with inhibitors of histone deacetylase (HDAC).
Jaebok Lee, Marc Diederich
wiley   +1 more source