Results 21 to 30 of about 7,033 (216)

Acid Ceramidase Deficiency: Bridging Gaps between Clinical Presentation, Mouse Models, and Future Therapeutic Interventions

Biomolecules, 2023
Farber disease (FD) and spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME) are ultra-rare, autosomal-recessive, acid ceramidase (ACDase) deficiency disorders caused by ASAH1 gene mutations.
Annie Kleynerman, J. Rybova, Mary Faber, W. McKillop, T. Levade, J. Medin   +5 more
semanticscholar   +1 more source

Digestion and Absorption of Milk Phospholipids in Newborns and Adults

Frontiers in Nutrition, 2021
Milk polar lipids provide choline, ethanolamine, and polyunsaturated fatty acids, which are needed for the growth and plasticity of the tissues in a suckling child.
Åke Nilsson, Rui-Dong Duan, Lena Ohlsson   +2 more
doaj   +1 more source

Exosomal miR-125b-5p derived from adipose-derived mesenchymal stem cells enhance diabetic hindlimb ischemia repair via targeting alkaline ceramidase 2

Journal of Nanobiotechnology, 2023
Introduction Ischemic diseases caused by diabetes continue to pose a major health challenge and effective treatments are in high demand. Mesenchymal stem cells (MSCs) derived exosomes have aroused broad attention as a cell-free treatment for ischemic ...
Jiahe Guo, Xiaofan Yang, Jing Chen, Cheng Wang, Yueming Sun, Chengqi Yan, Sen Ren, Hewei Xiong, Kaituo Xiang, Maojie Zhang, Chengcheng Li, Guoyong Jiang, Xuejiao Xiang, Gui Wan, Tao Jiang, Yu Kang, Xiang Xu, Zhenbing Chen, Wenqing Li   +18 more
semanticscholar   +1 more source

Inhibitory effect of Porphyromonas gingivalis‐derived phosphoethanolamine dihydroceramide on acid ceramidase expression in oral squamous cells

Journal of Cellular and Molecular Medicine, 2023
The maintenance of diminished acid ceramidase (ASAH1) gene expression leading to the accumulation of antiproliferative intracellular ceramides in oral squamous cell carcinoma (OSCC) has emerged as a prospective oral cancer therapeutic regimen.
Chiaki Yamada, Anny Ho, A. Nusbaum, R. Xu, M. E. Davey, F. Nichols, C. Mao, A. Movila   +7 more
semanticscholar   +1 more source

Targeting acid ceramidase enhances antitumor immune response in colorectal cancer

Journal of Advanced Research, 2023
Graphical ...
Yadu Vijayan, S. James, A. Viswanathan, J. Aparna, Anu Bindu, N. N. Namitha, Devasena Anantharaman, Manendra Babu Lankadasari, K. Harikumar   +8 more
semanticscholar   +1 more source

Acid ceramidase involved in pathogenic cascade leading to accumulation of α-synuclein in iPSC model of GBA1-associated Parkinson’s disease

Human Molecular Genetics, 2023
Bi-allelic mutations in GBA1, the gene that encodes β-glucocerebrosidase (GCase), cause Gaucher disease (GD), whereas mono-allelic mutations do not cause overt pathology.
M. Kumar, Manasa P. Srikanth, M. Deleidi, P. Hallett, O. Isacson, R. Feldman   +5 more
semanticscholar   +1 more source

Spinal muscular atrophy-like phenotype in a mouse model of acid ceramidase deficiency

Communications Biology, 2023
Mutations in ASAH1 have been linked to two allegedly distinct disorders: Farber disease (FD) and spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME).
Murtaza S. Nagree, J. Rybova, Annie Kleynerman, Carissa J Ahrenhoerster, Jennifer T. Saville, Tian-zhao Xu, Maxwell J. Bachochin, W. McKillop, M. Lawlor, A. Pshezhetsky, O. Isaeva, M. Budde, M. Fuller, J. Medin   +13 more
semanticscholar   +1 more source

Elusive Roles of the Different Ceramidases in Human Health, Pathophysiology, and Tissue Regeneration

Cells, 2020
Ceramide and sphingosine are important interconvertible sphingolipid metabolites which govern various signaling pathways related to different aspects of cell survival and senescence. The conversion of ceramide into sphingosine is mediated by ceramidases.
Carolina Duarte, Juliet Akkaoui, Chiaki Yamada, Anny Ho, Cungui Mao, Alexandru Movila   +5 more
doaj   +1 more source

Acid Ceramidase Inhibitor LCL-805 Antagonizes Akt Signaling and Promotes Iron-Dependent Cell Death in Acute Myeloid Leukemia

Cancers, 2023
Simple Summary The lysosomal lipid hydrolase acid ceramidase is upregulated in acute myeloid leukemia and promotes leukemic blast survival, underscoring its potential for therapeutic targeting.
J. Ung, Su-Fern Tan, Todd E Fox, Jeremy J. P. Shaw, Maansi Taori, Bethany J Horton, Upendarrao Golla, Arati Sharma, Z. Szulc, Hong-Gang Wang, C. E. Chalfant, M. Cabot, David F Claxton, T. Loughran, D. Feith   +14 more
semanticscholar   +1 more source

Upregulation of acid ceramidase contributes to tumor progression in tuberous sclerosis complex

JCI Insight, 2023
Tuberous sclerosis complex (TSC) is characterized by multisystem, low-grade neoplasia involving the lung, kidneys, brain, and heart. Lymphangioleiomyomatosis (LAM) is a progressive pulmonary disease affecting almost exclusively women.
A. Astrinidis, Chenggang Li, E. Zhang, Xueheng Zhao, Shuyang Zhao, M. Guo, Tasnim Olatoke, Ushodaya Mattam, Rong Huang, Alan G. Zhang, L. Pitstick, E. Kopras, N. Gupta, R. Jandarov, Eric P. Smith, E. Fugate, Diana Lindquist, M. Markiewski, M. Karbowniczek, K. Wikenheiser-Brokamp, K. Setchell, F. McCormack, Yan Xu, Jane J Yu   +23 more
semanticscholar   +1 more source