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Literature summary for 3.5.1.23 extracted from
- Purification and characterization of recombinant, human acid ceramidase. Catalytic reactions and interactions with acid sphingomyelinase (2003), J. Biol. Chem., 278, 32978-32986.
Application
| Application | Comment | Organism |
|---|---|---|
| medicine | patients with the lipid storage disorder, the Farber disease, have a marked AC deficiency | Homo sapiens |
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
| overexpression in CHO cells and in normal human skin fibroblasts | Homo sapiens |
KM Value [mM]
| KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|
| 0.389 | - |
N-lauroylsphingosine | pH 4.5, 37°C, recombinant AC expressed in CHO cells, 14C-labeled substrate | Homo sapiens |  |
| 0.4132 | - |
N-lauroylsphingosine | pH 4.5, 37°C, recombinant AC expressed in CHO cells, BODIPY-conjugated substrate | Homo sapiens | |
Localization
| Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|
| extracellular | the majority of the recombinant AC overexpressed in CHO cells is secreted into the culture media | Homo sapiens | - |
- |
Molecular Weight [Da]
| Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
|---|---|---|---|
| 13000 | - |
x * 13000 + x * 40000, alpha- and beta-subunit, recombinant AC from CHO cells contains two distinct beta-subunits likely due to C-terminal processing, SDS-PAGE | Homo sapiens |
| 40000 | - |
x * 13000 + x * 40000, alpha- and beta-subunit, recombinant AC from CHO cells contains two distinct beta-subunits likely due to C-terminal processing, SDS-PAGE | Homo sapiens |
| 150000 | - |
gel filtration | Homo sapiens |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| additional information | Homo sapiens | critical role of AC in the regulation of sphingolipid metabolism | ? | - |
? | |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
- |
- |
Posttranslational Modification
| Posttranslational Modification | Comment | Organism |
|---|---|---|
| glycoprotein | recombinant AC from CHO cells contains mostly high mannose-type oligosaccharides, beta-subunit is glycosylated | Homo sapiens |
| proteolytic modification | AC is first synthesized as 55 kDa precursor protein that is proteolytically processed | Homo sapiens |
Purification (Commentary)
| Purification (Comment) | Organism |
|---|---|
| recombinant AC expressed in CHO cells from the culture media, 58fold | Homo sapiens |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|
Specific Activity [micromol/min/mg]
| Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
|---|---|---|---|
| 0.0338 | - |
pH 4.5, 37°C, recombinant AC expressed in CHO cells, hydrolysis of N-lauroylsphingosine | Homo sapiens |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| additional information | critical role of AC in the regulation of sphingolipid metabolism | Homo sapiens | ? | - |
? | |
| N-acylsphingosine + H2O | AC catalyzes the hydrolysis of ceramide to sphingosine and fatty acid | Homo sapiens | a carboxylate + sphingosine | - |
r | |
| N-lauroylsphingosine + H2O | optimal substrate, AC also efficiently catalyzes ceramide synthesis | Homo sapiens | lauric acid + sphingosine | - |
r | |
Subunits
| Subunits | Comment | Organism |
|---|---|---|
| ? | x * 13000 + x * 40000, alpha- and beta-subunit, recombinant AC from CHO cells contains two distinct beta-subunits likely due to C-terminal processing, SDS-PAGE | Homo sapiens |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| AC | acid ceramidase | Homo sapiens |
| acid ceramidase | - |
Homo sapiens |
| N-acylsphingosine deacylase | - |
Homo sapiens |
Temperature Optimum [°C]
| Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
|---|---|---|---|
| 37 | - |
assay at | Homo sapiens |
pH Optimum
| pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
|---|---|---|---|
| 4.5 | - |
ceramidase reaction, distinct pH-optima for the degradative and synthetic reaction | Homo sapiens |
| 5.5 | 6 | ceramide synthesis reaction, distinct pH-optima for the degradative and synthetic reaction | Homo sapiens |
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