KEGG   ENZYME: 1.14.11.2Help
Entry
EC 1.14.11.2                Enzyme                                 
Name
procollagen-proline dioxygenase;
protocollagen hydroxylase;
proline hydroxylase;
proline,2-oxoglutarate 4-dioxygenase;
collagen proline hydroxylase;
hydroxylase, collagen proline;
peptidyl proline hydroxylase;
proline protocollagen hydroxylase;
proline, 2-oxoglutarate dioxygenase;
prolyl hydroxylase;
prolylprotocollagen dioxygenase;
prolylprotocollagen hydroxylase;
protocollagen proline 4-hydroxylase;
protocollagen proline dioxygenase;
protocollagen proline hydroxylase;
protocollagen prolyl hydroxylase;
prolyl 4-hydroxylase;
prolyl-glycyl-peptide, 2-oxoglutarate:oxygen oxidoreductase, 4-hydroxylating;
procollagen-proline 4-dioxygenase
Class
Oxidoreductases;
Acting on paired donors, with O2 as oxidant and incorporation or reduction of oxygen. The oxygen incorporated need not be derived from O2;
With 2-oxoglutarate as one donor, and incorporation of one atom of oxygen into each donor
BRITE hierarchy
Sysname
procollagen-L-proline,2-oxoglutarate:oxygen oxidoreductase (4-hydroxylating)
Reaction(IUBMB)
procollagen L-proline + 2-oxoglutarate + O2 = procollagen trans-4-hydroxy-L-proline + succinate + CO2 [RN:R03219]
Reaction(KEGG)
R03219;
(other) R01252
Show
Substrate
procollagen L-proline [CPD:C01078];
2-oxoglutarate [CPD:C00026];
O2 [CPD:C00007]
Product
procollagen trans-4-hydroxy-L-proline [CPD:C04398];
succinate [CPD:C00042];
CO2 [CPD:C00011]
Comment
Requires Fe2+ and ascorbate.
Pathway
Arginine and proline metabolism
Metabolic pathways
Orthology
K00472  
prolyl 4-hydroxylase
Genes
HSA: 
283208(P4HA3) 5033(P4HA1) 8974(P4HA2)
PTR: 
450891(P4HA1) 462047(P4HA2) 748564(P4HA3)
PPS: 
100976439(P4HA3) 100992298(P4HA1) 100993331(P4HA2)
GGO: 
101131612(P4HA1) 101133984(P4HA2) 101143792(P4HA3)
PON: 
100172658(P4HA1) 100433561(P4HA3) 100448172(P4HA2)
MCC: 
707157(P4HA2) 707927(P4HA1) 718872
MCF: 
MMU: 
18451(P4ha1) 18452(P4ha2) 320452(P4ha3)
RNO: 
360526(P4ha2) 361612(P4ha3) 64475(P4ha1)
CGE: 
HGL: 
101702867(P4ha1) 101711128(P4ha3) 101724121(P4ha2)
CFA: 
474672(P4HA2) 479242(P4HA1) 609381(P4HA3)
AML: 
100479558 100480425(P4HA1) 100480972(P4HA2)
FCA: 
101081160(P4HA3) 101094394(P4HA2) 101096846(P4HA1)
BTA: 
414348(P4HA3) 507327(P4HA2) 518288(P4HA1)
BOM: 
102274070(P4HA1) 102274248(P4HA2) 102280992(P4HA3)
PHD: 
102333228(P4HA1) 102335098(P4HA3) 102339818(P4HA2)
CHX: 
102179448(P4HA1) 102186234(P4HA3) 102189116(P4HA2)
SSC: 
100037299(P4HA1) 100623801(P4HA2) 100624543(P4HA3)
ECB: 
100063760(P4HA1) 100065260(P4HA3) 100072882
MYB: 
102242052(P4HA2) 102245712(P4HA3) 102255473(P4HA1)
MDO: 
100010507(P4HA1) 100013447 100018258
SHR: 
100913891 100921246(P4HA3) 100931276(P4HA2)
OAA: 
100077226(P4HA1) 100079170
GGA: 
416326(P4HA2) 419057(P4HA3) 423704(P4HA1)
MGP: 
100544911 100547385 100550944
TGU: 
100223373(P4HA3) 100226743(P4HA2) 100228350(P4HA1)
FAB: 
101807615(P4HA3) 101808225 101809059(P4HA1)
PHI: 
102110972(P4HA3) 102113563(P4HA2) 102114022(P4HA1)
APLA: 
101792390(P4HA2) 101799325(P4HA3) 101802328(P4HA1)
FPG: 
101913115(P4HA2) 101915502(P4HA1) 101921488(P4HA3)
FCH: 
102047744(P4HA2) 102052133(P4HA1) 102054422(P4HA3)
CLV: 
102092707(P4HA3) 102096249(P4HA1) 102096911(P4HA2)
ASN: 
102370647(P4HA3) 102373565(P4HA2) 102376142(P4HA1)
ACS: 
100565232 100567447
XLA: 
447527(p4ha2)
XTR: 
100490484 100492280(p4ha1) 100495824 493342(p4ha2)
DRE: 
100003675(p4ha1b) 373132(p4ha2) 556879 562774(p4ha1a) 563281(p4ha3)
TRU: 
101064031 101066030 101075755
MZE: 
101464180 101464219 101485561 101486537
OLA: 
101161986 101163841 101166436
XMA: 
102216605 102219518 102220364
LCM: 
102346232(P4HA2) 102360424(P4HA3) 102363342(P4HA1)
BFO: 
BRAFLDRAFT_59428 BRAFLDRAFT_84679
CIN: 
100180859 100182956 100184843 100185314
SPU: 
592929
DME: 
Dmel_CG11828 Dmel_CG1546(PH4alphaSG2) Dmel_CG15539 Dmel_CG18749 Dmel_CG31014(PH4alphaSG1) Dmel_CG31015(PH4alphaPV) Dmel_CG31016 Dmel_CG31017(PH4alphaNE3) Dmel_CG31022(PH4alphaEFB) Dmel_CG31524 Dmel_CG32199 Dmel_CG32201 Dmel_CG34345 Dmel_CG4174 Dmel_CG9698 Dmel_CG9720(PH4alphaNE2) Dmel_CG9726(PH4alphaMP) Dmel_CG9728(PH4alphaNE1)
DPO: 
Dpse_GA13747 Dpse_GA15938 Dpse_GA15939 Dpse_GA15946 Dpse_GA21971 Dpse_GA21989 Dpse_GA21991 Dpse_GA26861 Dpse_GA26871 Dpse_GA26875
DAN: 
Dana_GF10816 Dana_GF14452 Dana_GF22904 Dana_GF22909 Dana_GF22917 Dana_GF23317 Dana_GF23328 Dana_GF23330 Dana_GF23331 Dana_GF23629 Dana_GF23631
DER: 
Dere_GG11627 Dere_GG11753 Dere_GG11755 Dere_GG11756 Dere_GG11758 Dere_GG11766 Dere_GG11767 Dere_GG11918 Dere_GG11919 Dere_GG11927 Dere_GG11928 Dere_GG11929 Dere_GG11931 Dere_GG11932 Dere_GG13664 Dere_GG13666 Dere_GG15736
DPE: 
Dper_GL13463 Dper_GL13464 Dper_GL13472 Dper_GL13506 Dper_GL13507 Dper_GL13509 Dper_GL13994 Dper_GL14040 Dper_GL14042 Dper_GL14044 Dper_GL22882 Dper_GL24244
DSE: 
Dsec_GM12139 Dsec_GM12140 Dsec_GM12146 Dsec_GM12148 Dsec_GM12149 Dsec_GM12151 Dsec_GM12152 Dsec_GM12749 Dsec_GM12882 Dsec_GM12884 Dsec_GM12885 Dsec_GM12888 Dsec_GM12897 Dsec_GM12898 Dsec_GM14934 Dsec_GM14980 Dsec_GM14981 Dsec_GM16188 Dsec_GM23710
DSI: 
Dsim_GD12338 Dsim_GD14757 Dsim_GD14758 Dsim_GD16902 Dsim_GD16913 Dsim_GD16980 Dsim_GD16991 Dsim_GD17002 Dsim_GD17024 Dsim_GD17035 Dsim_GD18523 Dsim_GD21390 Dsim_GD21521 Dsim_GD21523 Dsim_GD21524 Dsim_GD21527 Dsim_GD21536 Dsim_GD21537 Dsim_GD23422
DWI: 
Dwil_GK10713 Dwil_GK13116 Dwil_GK13123 Dwil_GK13124 Dwil_GK13125 Dwil_GK14136 Dwil_GK14137 Dwil_GK14140 Dwil_GK14141 Dwil_GK14149
DYA: 
Dyak_GE10881 Dyak_GE10884 Dyak_GE10885 Dyak_GE10887 Dyak_GE10893 Dyak_GE10895 Dyak_GE14021 Dyak_GE19962 Dyak_GE22068 Dyak_GE23368 Dyak_GE23370 Dyak_GE23378 Dyak_GE23379 Dyak_GE23380 Dyak_GE23382 Dyak_GE23383 Dyak_GE23815
DGR: 
Dgri_GH12977 Dgri_GH14105 Dgri_GH14110 Dgri_GH14132 Dgri_GH14207 Dgri_GH17427 Dgri_GH17428
DMO: 
Dmoj_GI10637 Dmoj_GI10638 Dmoj_GI13597 Dmoj_GI22114 Dmoj_GI22756 Dmoj_GI22757 Dmoj_GI23043 Dmoj_GI24860 Dmoj_GI24861 Dmoj_GI24862
DVI: 
Dvir_GJ13934 Dvir_GJ22757 Dvir_GJ22759 Dvir_GJ22993 Dvir_GJ22994 Dvir_GJ24233 Dvir_GJ24502 Dvir_GJ24503 Dvir_GJ24504 Dvir_GJ24631
AGA: 
AgaP_AGAP000971 AgaP_AGAP004611
AAG: 
AaeL_AAEL005714 AaeL_AAEL007032 AaeL_AAEL007038
CQU: 
CpipJ_CPIJ017127 CpipJ_CPIJ017129 CpipJ_CPIJ017131
AME: 
408862(PH4alphaEFB)
NVI: 
100116138
TCA: 
657862 657945
BMOR: 
692683
API: 
100166186
PHU: 
Phum_PHUM445570
ISC: 
IscW_ISCW001441
CEL: 
CELE_F35G2.4(phy-2) CELE_Y47D3B.10(dpy-18)
CBR: 
CBG06252(Cbr-phy-2) CBG13195(Cbr-dpy-18)
BMY: 
Bm1_45455
LOA: 
LOAG_06927
TSP: 
Tsp_03346 Tsp_03348
SMM: 
Smp_068280
NVE: 
NEMVE_v1g120212 NEMVE_v1g120225 NEMVE_v1g195205
HMG: 
100207876 100211222 100214656
TAD: 
TRIADDRAFT_30039 TRIADDRAFT_59899
AQU: 
100634548
ATH: 
AT1G20270 AT2G17720 AT2G23096 AT2G43080(AT-P4H-1) AT3G06300(AT-P4H-2) AT3G28480 AT3G28490 AT4G33910 AT4G35810 AT4G35820 AT5G18900 AT5G66060
ALY: 
ARALYDRAFT_328172 ARALYDRAFT_472257 ARALYDRAFT_480667 ARALYDRAFT_481183 ARALYDRAFT_484624 ARALYDRAFT_488774 ARALYDRAFT_491287 ARALYDRAFT_497011 ARALYDRAFT_656411 ARALYDRAFT_664704 ARALYDRAFT_896741 ARALYDRAFT_903688
GMX: 
100527885 100775302 100776115 100783075 100796794 100798201 100805097 100806046 100820384 100820606
MTR: 
MTR_1g116810 MTR_2g020800 MTR_3g114670 MTR_4g095370 MTR_4g118560 MTR_5g022960 MTR_6g009440
CAM: 
101488382 101496423 101502651 101503898 101504842 101511375 101512678
FVE: 
101297607 101303432 101306377 101310176 101312187 101312625 101312919
CSV: 
101204265 101206818 101209446 101210382 101215570 101217481 101218235 101231596 101231616
RCU: 
RCOM_0174710 RCOM_0396560 RCOM_0679070 RCOM_0782750 RCOM_0853250 RCOM_0998510 RCOM_1599420
POP: 
POPTR_1085714 POPTR_1087308 POPTR_1105096 POPTR_585678 POPTR_593788 POPTR_801579 POPTR_830522 POPTR_908378
VVI: 
100241153 100242241 100244673 100245913 100252283 100252286 100256084
SLY: 
101244118 101244153 101246711 101254254 101259696 101262682 101265933 101267095
OSA: 
4334196 4334477 4335530 4339155 4342642 4348615 9270970
DOSA: 
Os03t0761900-01(Os03g0761900) Os03t0803500-01(Os03g0803500) Os04t0346000-01(Os04g0346000) Os05t0489100-01(Os05g0489100) Os07t0194500-01(Os07g0194500) Os10t0413500-01(Os10g0413500) Os10t0497800-01(Os10g0497800)
BDI: 
100830921 100831521 100832694 100834813 100835020 100835342 100843006
SBI: 
SORBI_01g004670(SORBIDRAFT_01g004670) SORBI_01g007280(SORBIDRAFT_01g007280) SORBI_01g018200(SORBIDRAFT_01g018200) SORBI_01g022150(SORBIDRAFT_01g022150) SORBI_02g005750(SORBIDRAFT_02g005750) SORBI_02g005760(SORBIDRAFT_02g005760) SORBI_04g038020(SORBIDRAFT_04g038020) SORBI_06g004550(SORBIDRAFT_06g004550) SORBI_08g016950(SORBIDRAFT_08g016950) SORBI_09g023860(SORBIDRAFT_09g023860)
ZMA: 
100193348 100193935(pco071536) 100274058 100282945(pco107217) 100283657(cl32377_1(260)) 100284871(pco093706) 100303994 100383727 100857080(P4H4) 100857083(P4H6)
SITA: 
101753221 101757367 101759577 101769812 101775858 101777428 101779189 101783088
SMO: 
SELMODRAFT_106693 SELMODRAFT_234144 SELMODRAFT_411114 SELMODRAFT_418273 SELMODRAFT_431327 SELMODRAFT_67344
PPP: 
PHYPADRAFT_115022 PHYPADRAFT_16503 PHYPADRAFT_168322 PHYPADRAFT_202199 PHYPADRAFT_5584 PHYPADRAFT_68659
CRE: 
CHLREDRAFT_111407 CHLREDRAFT_114525 CHLREDRAFT_122372 CHLREDRAFT_138462(PHX1) CHLREDRAFT_138508 CHLREDRAFT_143143
VCN: 
VOLCADRAFT_120451 VOLCADRAFT_62820 VOLCADRAFT_64439 VOLCADRAFT_80309 VOLCADRAFT_82641
OLU: 
OSTLU_37102 OSTLU_37480
OTA: 
Ot01g03290 Ot02g07180 Ot04g00140(P4HA1)
MIS: 
MICPUN_70761
MPP: 
MICPUCDRAFT_15413 MICPUCDRAFT_34636
CSL: 
COCSUDRAFT_25518 COCSUDRAFT_35772 COCSUDRAFT_47131
CVR: 
CHLNCDRAFT_28161 CHLNCDRAFT_35843 CHLNCDRAFT_49444
NCR: 
NCU00207
SMP: 
SMAC_05290
PAN: 
PODANSg4791
TTT: 
THITE_2118894
MTM: 
MYCTH_2060641
MGR: 
MGG_06278
FGR: 
FG05473.1
NHE: 
NECHADRAFT_71135
VAL: 
VDBG_09816
SSL: 
SS1G_08912
BFU: 
BC1G_10940
ANI: 
AN2851.2
NFI: 
NFIA_065320
AFM: 
AFUA_3G11980
AOR: 
AOR_1_1350154(AO090003000761)
ANG: 
ANI_1_2732024(An02g07870)
AFV: 
AFLA_030540
ACT: 
ACLA_039860
PCS: 
Pc18g00410
CIM: 
CIMG_09826
CPW: 
CPC735_022610
PNO: 
SNOG_03610
PTE: 
PTT_06584
ZTR: 
MYCGRDRAFT_33455
MBR: 
MONBRDRAFT_23906 MONBRDRAFT_37507
DDI: 
DDB_G0275385
DPP: 
DICPUDRAFT_151895
DFA: 
DFA_03579
TAN: 
TA04445
BEQ: 
BEWA_013750
TGO: 
TGME49_021270
PTI: 
PHATRDRAFT_13557 PHATRDRAFT_38694 PHATRDRAFT_48891 PHATRDRAFT_4950 PHATRDRAFT_5156 PHATRDRAFT_55162 PHATR_10655
TPS: 
THAPSDRAFT_18190 THAPSDRAFT_24375 THAPSDRAFT_24377 THAPSDRAFT_262637 THAPSDRAFT_263006 THAPSDRAFT_264010 THAPSDRAFT_264177 THAPSDRAFT_4571 THAPSDRAFT_5584 THAPSDRAFT_6480 THAPSDRAFT_8415 THAPSDRAFT_9519 THAPS_10787 THAPS_38110
PIF: 
PITG_04973 PITG_19004
EHX: 
EMIHUDRAFT_119786 EMIHUDRAFT_229551 EMIHUDRAFT_367381 EMIHUDRAFT_435815 EMIHUDRAFT_440074 EMIHUDRAFT_465463 EMIHUDRAFT_55595 EMIHUDRAFT_55676 EMIHUDRAFT_65594 EMIHUDRAFT_77912
GTT: 
GUITHDRAFT_114401 GUITHDRAFT_150687 GUITHDRAFT_44480 GUITHDRAFT_71994
XCC: 
XCC3429
XCB: 
XC_0735
XCV: 
XCV0752
XCP: 
XCR_3769
XAC: 
XAC0691
XAX: 
XACM_0696
XAO: 
XAC29_03515
XCI: 
XCAW_03890
PSU: 
Psesu_0952
PSD: 
DSC_10945
RHD: 
R2APBS1_0370
PRW: 
PsycPRwf_1859
SDE: 
Sde_3273 Sde_3700
AMC: 
MADE_1006225
AMAC: 
MASE_03040 MASE_05190
AMB: 
AMBAS45_03290
AMG: 
AMEC673_03255 AMEC673_05270
AMH: 
I633_05910
AMK: 
AMBLS11_03220 AMBLS11_05295
AMAA: 
amad1_05610
AMAL: 
I607_05385
AMAE: 
I876_05675
AMAO: 
I634_05700
AMAD: 
I636_05620
AMAI: 
I635_05585
AMAG: 
I533_05275
ALT: 
ambt_12960 ambt_14915
MMT: 
Metme_0168
TKM: 
TK90_2795
AFE: 
Lferr_2737
AFR: 
AFE_1210
AFI: 
Acife_1859
SAGA: 
M5M_16075
RSO: 
RSc2814
RSC: 
RCFBP_10646
RSL: 
RPSI07_0693
RSN: 
RSPO_c00694
RSM: 
CMR15_10601
RSE: 
F504_2741
RPI: 
Rpic_3059
RPF: 
Rpic12D_2649
REU: 
Reut_A2950
REH: 
H16_A3244(phy)
RME: 
Rmet_3098
CTI: 
RALTA_A2697
CNC: 
CNE_1c31800(phy)
BXE: 
Bxe_B0348 Bxe_B1199
BPH: 
Bphy_5329
BPY: 
Bphyt_5409
BUG: 
BC1001_3722
BGE: 
BC1002_3991 BC1002_4858
BGF: 
BC1003_3769
BGD: 
bgla_2g25050
BYI: 
BYI23_D000440 BYI23_D012160
BPX: 
BUPH_00288
POL: 
Bpro_3712
AAV: 
Aave_1171
AJS: 
Ajs_3293
DIA: 
Dtpsy_2646
AAA: 
Acav_1184
ACK: 
C380_07130
DAC: 
Daci_5284
DEL: 
DelCs14_1329
VAP: 
Vapar_1302 Vapar_3584
VPE: 
Varpa_1403 Varpa_4544
VPD: 
VAPA_1c13750 VAPA_1c37020
CTT: 
CtCNB1_0958
ADN: 
Alide_3198
ADK: 
Alide2_1249
RTA: 
Rta_28970
MPT: 
Mpe_A2091
CFU: 
CFU_2670
LCH: 
Lcho_2651
RGE: 
RGE_13160
BBT: 
BBta_1313
HNE: 
HNE_1861
NAR: 
Saro_1382
NPP: 
PP1Y_AT14227
SAL: 
Sala_1522
SWI: 
Swit_2573
SJP: 
SJA_C1-32480
SCH: 
Sphch_0026
SSY: 
SLG_08240 SLG_13730
ELI: 
ELI_02985 ELI_03845
BAN: 
BA_4459
BAR: 
GBAA_4459
BAT: 
BAS4138
BAH: 
BAMEG_4493
BAI: 
BAA_4476
BAX: 
H9401_4253
BAL: 
BACI_c42010
BCE: 
BC4233
BCA: 
BCE_4314
BCZ: 
BCZK3988
BCR: 
BCAH187_A4367
BCB: 
BCB4264_A4348
BCU: 
BCAH820_4256
BCG: 
BCG9842_B0892
BCQ: 
BCQ_4020
BCX: 
BCA_4345
BNC: 
BCN_4148
BCF: 
bcf_21065
BCER: 
BCK_13995
BTK: 
BT9727_3978
BTL: 
BALH_3835
BTB: 
BMB171_C3904
BTT: 
HD73_4540
BTC: 
CT43_CH4249
BTF: 
YBT020_20895
BTM: 
MC28_3528
BTG: 
BTB_c43770
BTI: 
BTG_28220
BTN: 
BTF1_19550
BTHT: 
H175_ch4319
BTHU: 
YBT1518_23380
BWE: 
BcerKBAB4_4090
BTY: 
Btoyo_1479
BMQ: 
BMQ_2841
BMD: 
BMD_2875
BMH: 
BMWSH_2348
BCO: 
Bcell_0583
BIF: 
N288_14065
HHD: 
HBHAL_1453
BBE: 
BBR47_52080
PJD: 
Pjdr2_0570
TCO: 
Theco_3434
SIV: 
SSIL_1645
AYM: 
YM304_21960
SYW: 
SYNW2355
SYD: 
Syncc9605_2496
SYE: 
Syncc9902_2168
HAO: 
PCC7418_0240
 » show all
Taxonomy
Reference
1  [PMID:4346946]
  Authors
Berg RA, Prockop DJ.
  Title
Affinity column purification of protocollagen proline hydroxylase from chick embryos and further characterization of the enzyme.
  Journal
J. Biol. Chem. 248 (1973) 1175-82.
  Organism
Rattus norvegicus [GN:rno]
Reference
2
  Authors
Hutton, J.J., Jr., Tappel, A.L. and Udenfriend, S.
  Title
Cofactor and substrate requirements of collagen proline hydroxylase.
  Journal
Arch. Biochem. Biophys. 118 (1967) 231-240.
Reference
3  [PMID:5046811]
  Authors
Kivirikko KI, Kishida Y, Sakakibara S, Prockop DJ.
  Title
Hydroxylation of (X-Pro-Gly)n by protocollagen proline hydroxylase. Effect of chain length, helical conformation and amino acid sequence in the substrate.
  Journal
Biochim. Biophys. Acta. 271 (1972) 347-56.
  Organism
Rattus norvegicus [GN:rno], Gallus gallus [GN:gga]
Reference
4
  Authors
Kivirikko, K.I. and Prockop, D.J.
  Title
Purification and partial characterization of the enzyme for the hydroxylation of proline in protocollogen.
  Journal
Arch. Biochem. Biophys. 118 (1967) 611-618.
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 
CAS: 
9028-06-2
DBGET integrated database retrieval system