Gene/Proteome Database (LMPD)
LMPD ID
LMP000475
Gene ID
Species
Homo sapiens (Human)
Gene Name
alkaline ceramidase 1
Gene Symbol
Synonyms
ALKCDase1; ASAH3
Alternate Names
alkaline ceramidase 1; alkCDase 1; alkaline CDase 1; acylsphingosine deacylase 3; N-acylsphingosine amidohydrolase 3; N-acylsphingosine amidohydrolase (alkaline ceramidase) 3
Chromosome
19
Map Location
19p13.3
EC Number
3.5.1.23
Summary
Ceramides are synthesized during epidermal differentiation and accumulate within the interstices of the stratum corneum, where they represent critical components of the epidermal permeability barrier. Excess cellular ceramide can trigger antimitogenic signals and induce apoptosis, and the ceramide metabolites sphingosine and sphingosine-1-phosphate (S1P) are important bioregulatory molecules. Ceramide hydrolysis in the nucleated cell layers regulates keratinocyte proliferation and apoptosis in response to external stress. Ceramide hydrolysis also occurs at the stratum corneum, releasing free sphingoid base that functions as an endogenous antimicrobial agent. ACER1 is highly expressed in epidermis and catalyzes the hydrolysis of very long chain ceramides to generate sphingosine (Houben et al., 2006 [PubMed 16477081]; Sun et al., 2008 [PubMed 17713573]).[supplied by OMIM, Jul 2010]
Orthologs
Proteins
| alkaline ceramidase 1 | |
|---|---|
| Refseq ID | NP_597999 |
| Protein GI | 19424128 |
| UniProt ID | Q8TDN7 |
| mRNA ID | NM_133492 |
| Length | 264 |
| RefSeq Status | VALIDATED |
| MPSIFAYQSSEVDWCESNFQYSELVAEFYNTFSNIPFFIFGPLMMLLMHPYAQKRSRYIYVVWVLFMIIGLFSMYFHMTLSFLGQLLDEIAILWLLGSGYSIWMPRCYFPSFLGGNRSQFIRLVFITTVVSTLLSFLRPTVNAYALNSIALHILYIVCQEYRKTSNKELRHLIEVSVVLWAVALTSWISDRLLCSFWQRIHFFYLHSIWHVLISITFPYGMVTMALVDANYEMPGETLKVRYWPRDSWPVGLPYVEIRGDDKDC | |
Gene Information
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0005783 | IDA:BHF-UCL | C | endoplasmic reticulum |
| GO:0005789 | TAS:Reactome | C | endoplasmic reticulum membrane |
| GO:0016021 | IEA:UniProtKB-KW | C | integral component of membrane |
| GO:0017040 | IDA:BHF-UCL | F | ceramidase activity |
| GO:0071633 | IDA:BHF-UCL | F | dihydroceramidase activity |
| GO:0030154 | IMP:BHF-UCL | P | cell differentiation |
| GO:0071277 | IDA:BHF-UCL | P | cellular response to calcium ion |
| GO:0046514 | ISS:BHF-UCL | P | ceramide catabolic process |
| GO:0008544 | IEP:BHF-UCL | P | epidermis development |
| GO:0030216 | IEP:BHF-UCL | P | keratinocyte differentiation |
| GO:0019216 | IEA:Ensembl | P | regulation of lipid metabolic process |
| GO:0010446 | IDA:BHF-UCL | P | response to alkaline pH |
| GO:0044281 | TAS:Reactome | P | small molecule metabolic process |
| GO:0030148 | IDA:BHF-UCL | P | sphingolipid biosynthetic process |
| GO:0006665 | ISS:BHF-UCL | P | sphingolipid metabolic process |
| GO:0046512 | IDA:BHF-UCL | P | sphingosine biosynthetic process |
KEGG Pathway Links
| KEGG Pathway ID | Description |
|---|---|
| hsa00600 | Sphingolipid metabolism |
BIOCYC Pathway Links
| BIOCYC Pathway ID | Description |
|---|---|
| PWY3DJ-11470 | sphingosine and sphingosine-1-phosphate metabolism |
REACTOME Pathway Links
| REACTOME Pathway ID | Description |
|---|---|
| REACT_115810 | Sphingolipid de novo biosynthesis |
Domain Information
InterPro Annotations
| Accession | Description |
|---|---|
| IPR008901 | Ceramidase |
UniProt Annotations
Entry Information
Comments
| Comment Type | Description |
|---|---|
| Catalytic Activity | N-acylsphingosine + H(2)O = a carboxylate + sphingosine. |
| Enzyme Regulation | Inhibited by sphingosine. |
| Function | Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid at an optimal pH of 8.0. Has a highly restricted substrate specificity for the natural stereoisomer of ceramide with D-erythro-sphingosine but not D-ribo- phytosphingosine or D-erythro-dihydrosphingosine as a backbone. May have a role in regulating the levels of bioactive lipids ceramide and sphingosine 1-phosphate, as well as complex sphingolipids (By similarity). |
| Similarity | Belongs to the alkaline ceramidase family. |
| Subcellular Location | Endoplasmic reticulum membrane {ECO |
| Tissue Specificity | Mainly expressed in epidermis. |
Identical and Related Proteins
Unique RefSeq proteins for LMP000475 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 19424128 | RefSeq | NP_597999 | 264 | alkaline ceramidase 1 |
Identical Sequences to LMP000475 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:19424128 | GenBank | AAI12125.1 | 264 | Alkaline ceramidase 1 [Homo sapiens] |
| GI:19424128 | GenBank | AAI12123.1 | 264 | Alkaline ceramidase 1 [Homo sapiens] |
| GI:19424128 | GenBank | EAW69104.1 | 264 | N-acylsphingosine amidohydrolase (alkaline ceramidase) 3, isoform CRA_a [Homo sapiens] |
| GI:19424128 | GenBank | AIC52990.1 | 264 | ACER1, partial [synthetic construct] |
| GI:19424128 | RefSeq | XP_004059888.1 | 264 | PREDICTED: alkaline ceramidase 1 [Gorilla gorilla gorilla] |
| GI:19424128 | SwissProt | Q8TDN7.1 | 264 | RecName: Full=Alkaline ceramidase 1; Short=AlkCDase 1; Short=Alkaline CDase 1; AltName: Full=Acylsphingosine deacylase 3; AltName: Full=N-acylsphingosine amidohydrolase 3 [Homo sapiens] |
Related Sequences to LMP000475 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:19424128 | GenBank | EHH29527.1 | 264 | Alkaline ceramidase 1 [Macaca mulatta] |
| GI:19424128 | RefSeq | XP_524068.2 | 264 | PREDICTED: alkaline ceramidase 1 [Pan troglodytes] |
| GI:19424128 | RefSeq | XP_003281615.1 | 264 | PREDICTED: alkaline ceramidase 1 [Nomascus leucogenys] |
| GI:19424128 | RefSeq | XP_003819378.1 | 264 | PREDICTED: alkaline ceramidase 1 [Pan paniscus] |
| GI:19424128 | RefSeq | XP_003914798.1 | 264 | PREDICTED: alkaline ceramidase 1 [Papio anubis] |
| GI:19424128 | RefSeq | XP_005587750.1 | 264 | PREDICTED: alkaline ceramidase 1 isoform X1 [Macaca fascicularis] |