Names & Taxonomy
- Uniprot ID:
- P13674
- Entry Name:
- P4HA1_HUMAN
- Status:
- reviewed
- Protein Names:
- Prolyl 4-hydroxylase subunit alpha-1 (4-PH alpha-1) (EC 1.14.11.2) (Procollagen-proline,2-oxoglutarate-4-dioxygenase subunit alpha-1)
- Gene Names:
- P4HA1 P4HA
- Gene Names Primary:
- P4HA1
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 534
- Sequence:
- MIWYILIIGILLPQSLAHPGFFTSIGQMTDLIHTEKDLVTSLKDYIKAEEDKLEQIKKWAEKLDRLTSTATKDPEGFVGHPVNAFKLMKRLNTEWSELENLVLKDMSDGFISNLTIQRQYFPNDEDQVGAAKALLRLQDTYNLDTDTISKGNLPGVKHKSFLTAEDCFELGKVAYTEADYYHTELWMEQALRQLDEGEISTIDKVSVLDYLSYAVYQQGDLDKALLLTKKLLELDPEHQRANGNLKYFEYIMAKEKDVNKSASDDQSDQKTTPKKKGVAVDYLPERQKYEMLCRGEGIKMTPRRQKKLFCRYHDGNRNPKFILAPAKQEDEWDKPRIIRFHDIISDAEIEIVKDLAKPRLRRATISNPITGDLETVHYRISKSAWLSGYENPVVSRINMRIQDLTGLDVSTAEELQVANYGVGGQYEPHFDFARKDEPDAFKELGTGNRIATWLFYMSDVSAGGATVFPEVGASVWPKKGTAVFWYNLFASGEGDYSTRHAACPVLVGNKWVSNKWLHERGQEFRRPCTLSELE
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Endoplasmic reticulum lumen.
Function
- Function:
- Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
- Catalytic Activity:
- L-proline- + 2-oxoglutarate + O(2) = trans-4-hydroxy-L-proline- + succinate + CO(2).
- Cofactor:
- COFACTOR: Name=Fe(2+); Xref=ChEBI:CHEBI:29033; ; Note=Binds 1 Fe(2+) ion per subunit.;; COFACTOR: Name=L-ascorbate; Xref=ChEBI:CHEBI:38290;
- Cross Reference Drug Bank:
- DB01275 DB00172 DB00139 DB00126
- Gene Ontology Go:
- endoplasmic reticulum
endoplasmic reticulum lumen
intracellular membrane-bounded organelle
membrane
mitochondrion
procollagen-proline 4-dioxygenase complex
iron ion binding
L-ascorbic acid binding
oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen
procollagen-proline 4-dioxygenase activity
collagen fibril organization
peptidyl-proline hydroxylation to 4-hydroxy-L-proline - Gene Ontology Biological Process:
- collagen fibril organization
peptidyl-proline hydroxylation to 4-hydroxy-L-proline - Gene Ontology Molecular Function:
- iron ion binding
L-ascorbic acid binding
oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen
procollagen-proline 4-dioxygenase activity - Gene Ontology Cellular Component:
- endoplasmic reticulum
endoplasmic reticulum lumen
intracellular membrane-bounded organelle
membrane
mitochondrion
procollagen-proline 4-dioxygenase complex - Keywords:
- 3D-structure
Alternative splicing
Complete proteome
Dioxygenase
Endoplasmic reticulum
Glycoprotein
Iron
Metal-binding
Oxidoreductase
Reference proteome
Signal
TPR repeat
Vitamin C - Interacts With:
- P42858