Prolyl 3-hydroxylase 1 (leucine-& proline-enriched proteoglycan 1, leprecan-1, growth suppressor 1, LEPRE1, GROS1, P3H1, PSEC0109)
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Function
- basement membrane-associated chondroitin sulfate proteoglycan
- prolyl 3-hydroxylase activity catalyzing posttranslational formation of 3-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens, especially collagen type 4 & collagen type 5
- role in secretory pathway of cells (putative)
- growth suppressive activity in fibroblasts
procollagen L-proline + 2-oxoglutarate + O2
<-->
procollagen trans-3-hydroxy-L-proline + succinate + CO2
Cofactor: Fe+2/Fe+3, ascorbate
Structure
- O-glycosylated; chondroitin sulfate
- belongs to the leprecan family
- contains 1 PKHD domain (prolyl/lysyl hydroxylase domain)
- contains 4 TPR repeats
Compartment
- endoplasmic reticulum, extracellular matrix
- secreted into extracellular matrix as chondroitin sulfate proteoglycan
Alternative splicing
named isoforms=3