Ceramidases
| Acid ceramidase | ||
|---|---|---|
| Properties of human protein | ||
| Mass / length primary structure | 374 amino acids | |
| Secondary to quaternary structure | α + β = (121 + 253) aa | |
| Precursor | (395 aa) | |
| Isoforms | 2 | |
| Identifier | ||
| Gene name | ASAH1 | |
| External IDs | ||
| Enzyme classification | ||
| EC, category | 3.5.1.23 , hydrolase | |
| MEROPS | C89.001 | |
| Response type | Hydrolysis of N-acylsphingosine | |
| Substrate | N-acylsphingosine + H 2 O | |
| Products | Sphingosine + fatty acid | |
| Occurrence | ||
| Exceptions | Eukaryotes , bacteria | |
Ceramidases are enzymes that catalyze the cleavage of the N- acyl radical from ceramides , but also the reverse reaction of acylation . They occur in eukaryotes , but also in bacteria . They are divided into acidic, neutral and alkaline ceramidases depending on the area of activity. In humans, the activities of two neutral, two alkaline and one acidic ceramidase have been proven.
Acid ceramidase is not found in bacteria and is localized within the cells in the lysosomes . It is produced as a preprotein with 395 amino acids and, after the signal peptide has been cleaved, it is cut into two subunits which together form the enzyme complex . Mutations in ASAH1 - Gen acid ceramidase can for Farber disease lead. Their expression is regulated via the ACTH signaling pathway .
literature
- E. Eliyahu, N. Shtraizent et al. a .: Acid ceramidase improves the quality of oocytes and embryos and the outcome of in vitro fertilization. In: The FASEB journal . Volume 24, Number 4, April 2010, pp. 1229-1238. doi : 10.1096 / fj.09-145508 . PMID 20007509 .
- E. Ruckhäberle, U. Holtrich u. a .: Acid ceramidase 1 expression correlates with a better prognosis in ER-positive breast cancer. In: Climacteric . Volume 12, Number 6, December 2009, pp. 502-513. doi : 10.3109 / 13697130902939913 . PMID 19905902 .
Individual evidence
- ↑ InterPro : IPR008901 Ceramidase (English)
- ↑ UniProt search result
- ↑ a b UniProt Q13510
- ↑ N. Shtraizent, E. Eliyahu et al. a .: Autoproteolytic cleavage and activation of human acid ceramidase. In: The Journal of biological chemistry . Volume 283, Number 17, April 2008, pp. 11253-11259. doi : 10.1074 / jbc.M709166200 . PMID 18281275 . PMC 2431059 (free full text).
- ↑ N. Lucki, MB Sewer: The cAMP-responsive element binding protein (CREB) regulates the expression of acid ceramidase (ASAH1) in H295R human adrenocortical cells. In: Biochimica et biophysica acta . Volume 1791, Number 8, August 2009, pp. 706-713. doi : 10.1016 / j.bbalip.2009.03.005 . PMID 19298866 .