Presently, 7 human ceramidases encoded by 7 distinct genes have been cloned: acid ceramidase (ASAH1) - cell survival neutral ceramidase (ASAH2, ASAH2B, ASAH2C) - protective against inflammatory cytokines alkaline ceramidase 1 (ACER1) - mediating cell differentiation by controlling the generation of SPH and S1P alkaline ceramidase 2 (ACER2) - important for cell proliferation and survival alkaline ceramidase 3 (ACER3) A deficiency in ASAH1 is associated with Farber disease. (wikipedia.org)
Variants in the ASAH1 gene lead to severe reduction in acid ceramidase, typically to below 10 percent of normal. (medlineplus.gov)
Alves MQ, Le Trionnaire E, Ribeiro I, Carpentier S, Harzer K, Levade T, Ribeiro MG. Molecular basis of acid ceramidase deficiency in a neonatal form of Farber disease: identification of the first large deletion in ASAH1 gene. (medlineplus.gov)
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. (bvsalud.org)
Acid ceramidase (ACDase) -mangel er et spektrum af forstyrrelser, der inkluderer en sjælden lysosomal opbevaringsforstyrrelse kaldet Farber sygdom (FD) og en sjælden epileptisk lidelse kaldet spinal muskelatrofi med progressiv myoklonisk epilepsi (SMA-PME). (biomedicalhouse.com)
Nylige studier har fremhævet mulige biomarkører for en effektiv diagnose af ACDase-mangel. (biomedicalhouse.com)
Syre ceramidase (ACDase), der først blev oprenset i 1963, katalyserer syntesen og nedbrydningen af ceramid til sphingosin og fedtsyre (4). (biomedicalhouse.com)
I 1972 konstaterede Sugita og kolleger, at ACDase-aktivitet ikke var påviselig i post-mortem-væv fra en FD-patient (5). (biomedicalhouse.com)
In response to aging, the amount of ceramides increases in the cytoplasm of cells in conjunction with the diminished levels of acid ceramidase (ACdase), a lipid hydrolase that degrades ceramides. (nova.edu)
Acid ceramidase expression2
Inhibitory effect of Porphyromonas gingivalis-derived phosphoethanolamine dihydroceramide on acid ceramidase expression in oral squamous cells. (bvsalud.org)
Altogether, these data indicated that PEDHC produced by P. gingivalis inhibits acid ceramidase expression, promotes intracellular ceramide accumulation and suppresses the survival and migration of OSCC cells in vitro . (bvsalud.org)
Acid ceramidase deficiency: Farber disease and SMA-PME. (medlink.com)
Deacylase1
Ceramidase (EC 3.5.1.23, acylsphingosine deacylase, glycosphingolipid ceramide deacylase) is an enzyme which cleaves fatty acids from ceramide, producing sphingosine (SPH) which in turn is phosphorylated by a sphingosine kinase to form sphingosine-1-phosphate (S1P). (wikipedia.org)
Farber offentliggjorde senere en sagsrekke med tre patienter i 1952, som en transaktion for det 62. (biomedicalhouse.com)
Selvom Farber påviste en stigning i lipider i sine tidlige biokemiske undersøgelser, blev det vigtigste lipid, der ophobes ved Farber sygdom (FD), dvs. (biomedicalhouse.com)
Neutral4
Human neutral ceramidase (nCDase) is an enzyme that plays a critical role in colon cancer and there are currently no potent or clinically effective inhibitors for nCDase reported to date. (wikipedia.org)
Ceramidases are classified as acidic, neutral or basic according to the optimal pH with which they function. (nih.gov)
In the lysosomes, both active substances also suppress the activity of an enzyme group, the acid ceramidases. (news-medical.net)
The enzyme ceramidase is a new, completely unexpected target structure for antiviral therapy,' says Professor Jochen Bodem from the JMU Institute of Virology and Immunobiology. (news-medical.net)
Lead Optimization of Benzoxazolone Carboxamides as Orally Bioavailable and CNS Penetrant Acid Ceramidase Inhibitors. (nih.gov)
Protein1
Schematic of the acid ceramidase protein with indications of the corresponding numbers of mutations for each part. (medlink.com)
Evidence1
This study provides evidence that murine acid ceramidase (Ac) is required for normal erythropoiesis and the development of rodent malaria. (elifesciences.org)
Family1
In only one family of cysteine peptidases, is the role of the general base assigned to a residue other than a histidine: in peptidases from family C89 (acid ceramidase) an arginine is the general base. (embl.de)
Activity1
It is unclear whether the level of acid ceramidase activity is related to the severity of the disorder. (medlineplus.gov)
Alkaline5
Presently, 7 human ceramidases encoded by 7 distinct genes have been cloned: acid ceramidase (ASAH1) - cell survival neutral ceramidase (ASAH2, ASAH2B, ASAH2C) - protective against inflammatory cytokines alkaline ceramidase 1 (ACER1) - mediating cell differentiation by controlling the generation of SPH and S1P alkaline ceramidase 2 (ACER2) - important for cell proliferation and survival alkaline ceramidase 3 (ACER3) A deficiency in ASAH1 is associated with Farber disease. (wikipedia.org)
4. Role of alkaline ceramidases in the generation of sphingosine and its phosphate in erythrocytes. (nih.gov)
12. Deficiency of the alkaline ceramidase ACER3 manifests in early childhood by progressive leukodystrophy. (nih.gov)
16. Alkaline ceramidase 2 is essential for the homeostasis of plasma sphingoid bases and their phosphates. (nih.gov)
Whereas the inhibitor D-MAPP potently inhibits alkaline ceramidase (IC50 approximately 5μM) N-oleoylethanolamine shows only slight inhibitory characteristics towards this ceramidase.2 N-Oleoylethanolamine is chemically related to the endocannabinoid anandamide, although it is not a cannabinoid itself. (matreya.com)
Ceramide8
Ceramidase (EC 3.5.1.23, acylsphingosine deacylase, glycosphingolipid ceramide deacylase) is an enzyme which cleaves fatty acids from ceramide, producing sphingosine (SPH) which in turn is phosphorylated by a sphingosine kinase to form sphingosine-1-phosphate (S1P). (wikipedia.org)
Adiponectin receptors (ADIPORs) are integral membrane proteins that control glucose and lipid metabolism by mediating, at least in part, a cellular ceramidase activity that catalyses the hydrolysis of ceramide to produce sphingosine and a free fatty acid (FFA). (nih.gov)
Acid ceramidase (AC) is a lysosomal cysteine hydrolase that catalyzes the conversion of ceramide into fatty acid and sphingosine. (unipi.it)
To better understand the coexistence of acyl-CoA dependent ceramide synthases and ceramidases in the ER we investigated the membrane topology of Ypc1p by probing cysteine accessibility of natural and substituted cysteines with membrane non-permeating mass-tagged probes. (unifr.ch)
Altogether, these data indicated that PEDHC produced by P. gingivalis inhibits acid ceramidase expression, promotes intracellular ceramide accumulation and suppresses the survival and migration of OSCC cells in vitro . (bvsalud.org)
To elucidate the mechanisms that are involved in the decrease of ceramide levels in atopic dry skin and in aged skin, we examined both the activities of beta-glucocerebrosidase, which is a major enzyme in ceramide production, and of ceramidase, which is an essential enzyme in ceramide degradation, in the stratum corneum of atopic dry skin and aged skin. (medicaljournals.se)
The pregnancy was terminated at 22 weeks' gestation and the diagnosis was confirmed by the demonstration of considerably elevated renal and hepatic ceramide concentrations and severe reduction of ceramidase activity in fetal brain and cultured fibroblasts. (johnshopkins.edu)
Ceramidases (EC 3.5.1.23), such as ASAH2, catalyze hydrolysis of the N-acyl linkage of ceramide, a second messenger in a variety of cellular events, to produce sphingosine. (utsouthwestern.edu)
Acid ceramidase inhibitor1
LCL521 known as an Stigmasterol (Stigmasterin) acid ceramidase inhibitor is worthy of detailed investigation. (immune-source.com)
Sphingolipid Metabolism1
10. Ceramidases, roles in sphingolipid metabolism and in health and disease. (nih.gov)
Pfam1
The data allow inferring that all amino acids of Ypc1p that are conserved in the pfam PF05875 ceramidase motif and the CREST superfamily are located in or near the ER lumen. (unifr.ch)
Cellular1
E2 binds to the protein acid ceramidase, which sets off a chain of cellular processes that suppress angiogenesis. (yonsei.ac.kr)
Gene2
In May, Xuntian Jiang, PhD and colleagues had their research titled "CRISPR-Cas9 Knock-In of T513M and G41S Mutations in the Murine β-Galactosyl-Ceramidase Gene Re-capitulates Early-Onset and Adult-Onset Forms of Krabbe Disease," published in "Frontiers in Molecular Neuroscience. (wustl.edu)
and Bongarzone, Ernesto R, "CRISPR-Cas9 knock-in of T513M and G41S mutations in the murine β-galactosyl-ceramidase gene re-capitulates early-onset and adult-onset forms of Krabbe Disease. (wustl.edu)
Production1
The production involving anti-ceramidase pro-apoptocic ingredient (LCL385) provides an possibility to check in vivo the particular regards between hippocampal apopotosis and also figured out helplessness. (pdgfrsignaling.com)
Cells2
The overexpressed acid ceramidase was widely discovered on cancer cells, inducing apoptotic resistance on them.125 Therefore. (immune-source.com)
Inhibitory effect of Porphyromonas gingivalis-derived phosphoethanolamine dihydroceramide on acid ceramidase expression in oral squamous cells. (bvsalud.org)