Lysosomal protective protein
| Lysosomal protective protein | ||
|---|---|---|
|
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| Ribbon model of the PPCA dimer, according to 1IVY | ||
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Existing structural data : 1IVY , 3BP4 , 3BP7 , 3BXN , 4AZ0 , 4AZ3 , 4CI9 , 4CIA , 4CIB , 4MWS , 4MWT |
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| Properties of human protein | ||
| Mass / length primary structure | 452 amino acids | |
| Secondary to quaternary structure | 20 kDa + 32 kDa | |
| Precursor | (52 kDa) | |
| Identifier | ||
| Gene names | CTSA GLB2; GSL; NGBE; PPCA; PPGB | |
| External IDs | ||
| Enzyme classification | ||
| EC, category | 3.4.16.5 , serine protease | |
| MEROPS | S10.002 | |
| Response type | Hydrolysis of terminal amino acids of proteins | |
| Substrate | R-Xaa-Yaa + H 2 O | |
| Products | R-Xaa + Yaa | |
| Occurrence | ||
| Parent taxon | multicellular animals | |
| Orthologue | ||
| human | House mouse | |
| Entrez | 5476 | 19025 |
| Ensemble | ENSG00000064601 | ENSMUSG00000017760 |
| UniProt | P10619 | P16675 |
| Refseq (mRNA) | NM_000308 | NM_001038492 |
| Refseq (protein) | NP_000299 | NP_001033581 |
| Gene locus | Chr 20: 45.89 - 45.9 Mb | Chr 2: 164.83 - 164.84 Mb |
| PubMed search | 5476 |
19025
|
The lysosomal protective protein (PPCA) (also: cathepsin A or serine carboxypeptidase A ) is a protein which, as a dimer, binds to lysosomal enzymes ( β-galactosidases and neuraminidases ) and stabilizes them. It is therefore essential for the breakdown of glycosphingolipids . In addition, PPCA acts as a peptidase , which is able to separate a wide range of terminal amino acids from proteins. This exopeptidase activity is found in all animals and is possibly part of the antigen presentation in B cells . In humans cause mutations in the CTSA - gene for rare inherited Galactosialidosis .
Inhibitors of protease activity are antipain , ebelactone A, Piperastatin A .
literature
- M. Hiraiwa: Cathepsin A / protective protein: an unusual lysosomal multifunctional protein. In: Cellular and molecular life sciences: CMLS. Volume 56, Numbers 11-12, December 1999, pp. 894-907, ISSN 1420-682X . PMID 11212324 . (Review).
- V. Seyrantepe, A. Hinek et al. a .: Enzymatic activity of lysosomal carboxypeptidase (cathepsin) A is required for proper elastic fiber formation and inactivation of endothelin-1. In: Circulation. Volume 117, Number 15, April 2008, pp. 1973-1981, ISSN 1524-4539 . doi : 10.1161 / CIRCULATIONAHA.107.733212 . PMID 18391110 .
- Pshezhetsky, AV: Lysosomal carboxypeptidase A. In: Handbook of Proteolytic Enzymes, 2 edn (Ed .: Barrett, AJ, Rawlings, ND & Woessner, JF), pp. 1923–1929, Elsevier, London (2004)
Individual evidence
- ↑ UniProt P10619
- ↑ EJ Bonten, Y. Campos et al. a .: Heterodimerization of the sialidase NEU1 with the chaperone protective protein / cathepsin A prevents its premature oligomerization. In: The Journal of biological chemistry. Volume 284, Number 41, October 2009, pp. 28430-28441. doi : 10.1074 / jbc.M109.031419 . PMID 19666471 . PMC 2788892 (free full text).
- ↑ M. Reich, KD Spindler a. a .: Cathepsin A is expressed in primary human antigen-presenting cells. In: Immunology letters. Volume 128, Number 2, February 2010, pp. 143-147, ISSN 1879-0542 . doi : 10.1016 / j.imlet.2009.11.010 . PMID 19954752 .