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prolyl 3-hydroxylase 2 (leprecan-like protein 1, myxoid liposarcoma-associated protein 4, LEPREL1, MLAT4, P3H2)
Function:
- has prolyl 3-hydroxylase activity
- catalyzes posttranslational formation of 3-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens, especially collagen type 4 & collagen type 5
procollagen L-proline + 2-oxoglutarate + O2
procollagen trans-3-hydroxy-L-proline + succinate + CO2
Cofactor: Fe+2/Fe+3, ascorbate
Structure:
- belongs to the leprecan family
- contains 1 PKHD domain (prolyl/lysyl hydroxylase domain)
- contains 4 TPR repeats
Compartment: endoplasmic reticulum, Golgi
Expression:
- expressed in heart, placenta, lung, liver, skeletal muscle & kidney
- expression localized to the epithelia of bile ducts & to the sacroplasm of heart muscle & skeletal muscle
- in the pancreas, localized to a subpopulation of Langerhans islet cells
- in the salivary gland, expressed in acinar cells
General
glycoprotein
hydroxylase; monooxygenase
metalloprotein
Properties
SIZE: MW = 81 kD
entity length = 708 aa
COMPARTMENT: golgi
endoplasmic reticulum
MOTIF: signal sequence {1-24}
tetratricopeptide repeat {44-77}
proline-rich region
SITE: 93-99
MOTIF: proline residue (SEVERAL)
tetratricopeptide repeat {148-181}
tetratricopeptide repeat {210-243}
tetratricopeptide repeat {306-339}
N-glycosylation site {N449}
PKHD {462-670}
MOTIF: N-glycosylation site {N549}
Iron [Fe]-binding site
SITE: 580-580
Iron [Fe]-binding site
SITE: 582-582
Iron [Fe]-binding site
SITE: 652-652
arginine residue {R662}
ER retention {705-708}
Database Correlations
UniProt Q8IVL5
PFAM correlations
ENZYME 1.14.11.7
References
UniProt :accession Q8IVL5