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Prolyl 3-hydroxylase 3 (leprecan-like protein 2, protein B, LEPREL2, P3H3)
Function:
- 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
Alternative splicing: named isoforms=2
Expression:
- weak expression in heart, lung, ovary, skeletal muscle
General
glycoprotein
hydroxylase; monooxygenase
metalloprotein
Properties
SIZE: MW = 82 kD
entity length = 736 aa
COMPARTMENT: endoplasmic reticulum
MOTIF: signal sequence {1-20}
tetratricopeptide repeat {37-70}
tetratricopeptide repeat {154-187}
MOTIF: alanine-rich region {173-177}
MOTIF: alanine residue (SEVERAL)
tetratricopeptide repeat {216-249}
glutamate-rich region {267-270}
MOTIF: glutamate residue (SEVERAL)
tetratricopeptide repeat {316-349}
MOTIF: N-glycosylation site {N331}
N-glycosylation site {N462}
PKHD {464-674}
MOTIF: Iron [Fe]-binding site
SITE: 584-584
Iron [Fe]-binding site
SITE: 586-586
Iron [Fe]-binding site
SITE: 656-656
arginine residue {R666}
coiled coil {681-709}
MOTIF: glutamate-rich region {696-704}
MOTIF: glutamate residue (SEVERAL)
ER retention {733-736}
Database Correlations
UniProt Q8IVL6
Pfam PF03171
References
UniProt :accession Q8IVL6