Procollagen peptidase (EC 3.4.24.14, procollagen N-terminal peptidase, procollagen aminopeptidase, aminoprocollagen peptidase, aminoterminal procollagen peptidase, procollagen aminoterminal protease, procollagen N-terminal proteinase, type I/II procollagen N-proteinase, type III procollagen) is an endopeptidase involved in the processing of collagen. The proteases removes the terminal peptides of the procollagen. Deficiency of these enzymes leads to dermatosparaxisorEhlers–Danlos syndrome.[1]
| procollagen (type III) N-endopeptidase | |||||||
|---|---|---|---|---|---|---|---|
| Identifiers | |||||||
| Symbol | PCOLN3 | ||||||
| Alt. symbols | PRSM1 | ||||||
| NCBI gene | 5119 | ||||||
| HGNC | 8740 | ||||||
| OMIM | 164010 | ||||||
| RefSeq | NM_002768 | ||||||
| UniProt | Q9HD42 | ||||||
| Other data | |||||||
| EC number | 3.4.24.14 | ||||||
| Locus | Chr. 16 q24.3 | ||||||
| |||||||
The enzyme is present in the skin of rats and humans.[2]
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