| Identification |
|---|
| HMDB Protein ID
| CDBP04300 |
| Secondary Accession Numbers
| Not Available |
| Name
| Prolyl 4-hydroxylase subunit alpha-3 |
| Description
| Not Available |
| Synonyms
|
- 4-PH alpha-3
- Procollagen-proline,2-oxoglutarate-4-dioxygenase subunit alpha-3
|
| Gene Name
| P4HA3 |
| Protein Type
| Enzyme |
| Biological Properties |
|---|
| General Function
| Involved in oxidoreductase activity |
| Specific Function
| Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
|
| GO Classification
|
| Cellular Component |
| endoplasmic reticulum lumen |
| Component |
| endoplasmic reticulum |
| organelle |
| membrane-bounded organelle |
| intracellular membrane-bounded organelle |
| Function |
| peptidyl-proline dioxygenase activity |
| procollagen-proline dioxygenase activity |
| procollagen-proline 4-dioxygenase activity |
| l-ascorbic acid binding |
| iron ion binding |
| oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen |
| vitamin binding |
| oxidoreductase activity |
| ion binding |
| cation binding |
| metal ion binding |
| binding |
| catalytic activity |
| oxidoreductase activity, acting on single donors with incorporation of molecular oxygen |
| oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen |
| transition metal ion binding |
| oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, 2-oxoglutarate as one donor, and incorporation of one atom each of oxygen into both donors |
| Molecular Function |
| oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen |
| procollagen-proline 4-dioxygenase activity |
| L-ascorbic acid binding |
| iron ion binding |
| Process |
| metabolic process |
| oxidation reduction |
|
| Cellular Location
|
- Endoplasmic reticulum lumen (Probable)
|
| Pathways
|
| Name | SMPDB/Pathwhiz | KEGG | | Arginine and Proline Metabolism |    |  | | Prolidase Deficiency (PD) |    | Not Available | | Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency) |    | Not Available | | Hyperprolinemia Type II |    | Not Available | | Hyperprolinemia Type I |    | Not Available |
|
| Gene Properties |
|---|
| Chromosome Location
| 11 |
| Locus
| 11q13.4 |
| SNPs
| P4HA3 |
| Gene Sequence
|
>1635 bp
ATGGGTCCTGGGGCGCGGCTGGCGGCGCTGCTGGCGGTGCTGGCGCTCGGGACAGGAGAC
CCAGAAAGGGCTGCGGCTCGGGGCGACACGTTCTCGGCGCTGACCAGCGTGGCGCGCGCC
CTGGCGCCCGAGCGCCGGCTGCTGGGGCTGCTGAGGCGGTACCTGCGCGGGGAGGAGGCG
CGGCTGCGGGACCTGACTAGATTCTACGACAAGGTACTTTCTTTGCATGAGGATTCAACA
ACCCCTGTGGCTAACCCTCTGCTTGCATTTACTCTCATCAAACGCCTGCAGTCTGACTGG
AGGAATGTGGTACATAGTCTGGAGGCCAGTGAGAACATCCGAGCTCTGAAGGATGGCTAT
GAGAAGGTGGAGCAAGACCTTCCAGCCTTTGAGGACCTTGAGGGAGCAGCAAGGGCCCTG
ATGCGGCTGCAGGACGTGTACATGCTCAATGTGAAAGGCCTGGCCCGAGGTGTCTTTCAG
AGAGTCACTGGCTCTGCCATCACTGACCTGTACAGCCCCAAACGGCTCTTTTCTCTCACA
GGGGATGACTGCTTCCAAGTTGGCAAGGTGGCCTATGACATGGGGGATTATTACCATGCC
ATTCCATGGCTGGAGGAGGCTGTCAGTCTCTTCCGAGGATCTTACGGAGAGTGGAAGACA
GAGGATGAGGCAAGTCTAGAAGATGCCTTGGATCACTTGGCCTTTGCTTATTTCCGGGCA
GGAAATGTTTCGTGTGCCCTCAGCCTCTCTCGGGAGTTTCTTCTCTACAGCCCAGATAAT
AAGAGGATGGCCAGGAATGTCTTGAAATATGAAAGGCTCTTGGCAGAGAGCCCCAACCAC
GTGGTAGCTGAGGCTGTCATCCAGAGGCCCAATATACCCCACCTGCAGACCAGAGACACC
TACGAGGGGCTATGTCAGACCCTGGGTTCCCAGCCCACTCTCTACCAGATCCCTAGCCTC
TACTGTTCCTATGAGACCAATTCCAACGCCTACCTGCTGCTCCAGCCCATCCGGAAGGAG
GTCATCCACCTGGAGCCCTACATTGCTCTCTACCATGACTTCGTCAGTGACTCAGAGGCT
CAGAAAATTAGAGAACTTGCAGAACCATGGCTACAGAGGTCAGTGGTGGCATCAGGGGAG
AAGCAGTTACAAGTGGAGTACCGCATCAGCAAAAGTGCCTGGCTGAAGGACACTGTTGAC
CCAAAACTGGTGACCCTCAACCACCGCATTGCTGCCCTCACAGGCCTTGATGTCCGGCCT
CCCTATGCAGAGTATCTGCAGGTGGTGAACTATGGCATCGGAGGACACTATGAGCCTCAC
TTTGACCATGCTACGTCACCAAGCAGCCCCCTCTACAGAATGAAGTCAGGAAACCGAGTT
GCAACATTTATGATCTATCTGAGCTCGGTGGAAGCTGGAGGAGCCACAGCCTTCATCTAT
GCCAACCTCAGCGTGCCTGTGGTTAGGAATGCAGCACTGTTTTGGTGGAACCTGCACAGG
AGTGGTGAAGGGGACAGTGACACACTTCATGCTGGCTGTCCTGTCCTGGTGGGAGATAAG
TGGGTGGCCAACAAGTGGATACATGAGTATGGACAGGAATTCCGCAGACCCTGCAGCTCC
AGCCCTGAAGACTGA
|
| Protein Properties |
|---|
| Number of Residues
| 544 |
| Molecular Weight
| 61125.675 |
| Theoretical pI
| 6.492 |
| Pfam Domain Function
|
|
| Signals
|
Not Available
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
>Prolyl 4-hydroxylase subunit alpha-3
MGPGARLAALLAVLALGTGDPERAAARGDTFSALTSVARALAPERRLLGLLRRYLRGEEA
RLRDLTRFYDKVLSLHEDSTTPVANPLLAFTLIKRLQSDWRNVVHSLEASENIRALKDGY
EKVEQDLPAFEDLEGAARALMRLQDVYMLNVKGLARGVFQRVTGSAITDLYSPKRLFSLT
GDDCFQVGKVAYDMGDYYHAIPWLEEAVSLFRGSYGEWKTEDEASLEDALDHLAFAYFRA
GNVSCALSLSREFLLYSPDNKRMARNVLKYERLLAESPNHVVAEAVIQRPNIPHLQTRDT
YEGLCQTLGSQPTLYQIPSLYCSYETNSNAYLLLQPIRKEVIHLEPYIALYHDFVSDSEA
QKIRELAEPWLQRSVVASGEKQLQVEYRISKSAWLKDTVDPKLVTLNHRIAALTGLDVRP
PYAEYLQVVNYGIGGHYEPHFDHATSPSSPLYRMKSGNRVATFMIYLSSVEAGGATAFIY
ANLSVPVVRNAALFWWNLHRSGEGDSDTLHAGCPVLVGDKWVANKWIHEYGQEFRRPCSS
SPED
|
| External Links |
|---|
| GenBank ID Protein
| 36962719 |
| UniProtKB/Swiss-Prot ID
| Q7Z4N8 |
| UniProtKB/Swiss-Prot Entry Name
| P4HA3_HUMAN |
| PDB IDs
|
Not Available |
| GenBank Gene ID
| AY313448 |
| GeneCard ID
| P4HA3 |
| GenAtlas ID
| P4HA3 |
| HGNC ID
| HGNC:30135 |
| References |
|---|
| General References
| Not Available |