| Post Translational Modifications | N-glycosylated. Proteolytically cleaved into two chains alpha and beta that remain associated via a disulfide bond. Cleavage gives rise to a conformation change that activates the enzyme. The same catalytic Cys residue mediates the autoproteolytic cleavage and subsequent hydrolysis of lipid substrates. The beta chain may undergo an additional C-terminal processing. |
| Function | Lysosomal ceramidase that hydrolyzes sphingolipid ceramides into sphingosine and free fatty acids at acidic pH. Ceramides, sphingosine, and its phosphorylated form sphingosine-1-phosphate are bioactive lipids that mediate cellular signaling pathways regulating several biological processes including cell proliferation, apoptosis and differentiation. Has a higher catalytic efficiency towards C12-ceramides versus other ceramides. Also catalyzes the reverse reaction allowing the synthesis of ceramides from fatty acids and sphingosine. For the reverse synthetic reaction, the natural sphingosine D-erythro isomer is more efficiently utilized as a substrate compared to D-erythro-dihydrosphingosine and D-erythro-phytosphingosine, while the fatty acids with chain lengths of 12 or 14 carbons are the most efficiently used. Has also an N-acylethanolamine hydrolase activity. By regulating the levels of ceramides, sphingosine and sphingosine-1-phosphate in the epidermis, mediates the calcium-induced differentiation of epidermal keratinocytes. Also indirectly regulates tumor necrosis factor/TNF-induced apoptosis. By regulating the intracellular balance between ceramides and sphingosine, in adrenocortical cells, probably also acts as a regulator of steroidogenesis. Isoform 2: May directly regulate steroidogenesis by binding the nuclear receptor NR5A1 and negatively regulating its transcriptional activity. |
| Protein Name | Acid CeramidaseAcAcdaseAcid CdaseAcylsphingosine DeacylaseGlycosylceramide DeacylaseN-Acylethanolamine Hydrolase Asah1N-Acylsphingosine AmidohydrolasePutative 32 Kda Heart ProteinPhp32 Cleaved Into - Acid Ceramidase Subunit Alpha - Acid Ceramidase Subunit Beta |
| Database Links | Reactome: R-HSA-6798695Reactome: R-HSA-9840310Reactome: R-HSA-9857377 |
| Cellular Localisation | LysosomeSecretedSecretion Is Extremely Low And Localization To Lysosomes Is Mannose-6-Phosphate Receptor-DependentIsoform 2: NucleusCytoplasmA Localization To The Nucleus And The Cytoplasm Has Also Been Reported For Asah1Most Probably For Isoforms Devoid Of A Signal Peptide |
| Alternative Protein Names | Acid Ceramidase proteinAc proteinAcdase proteinAcid Cdase proteinAcylsphingosine Deacylase proteinGlycosylceramide Deacylase proteinN-Acylethanolamine Hydrolase Asah1 proteinN-Acylsphingosine Amidohydrolase proteinPutative 32 Kda Heart Protein proteinPhp32 Cleaved Into - Acid Ceramidase Subunit Alpha - Acid Ceramidase Subunit Beta proteinASAH1 proteinASAH proteinHSD-33 proteinHSD33 protein |
Information sourced from Uniprot.org
