KEGG   ENZYME: 1.14.11.2Help
Entry
EC 1.14.11.2                Enzyme                                 

Name
procollagen-proline 4-dioxygenase;
P4HA (gene name);
P4HB (gene name);
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 (ambiguous)
Class
Oxidoreductases;
Acting on paired donors, with incorporation or reduction of molecular oxygen;
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.The enzyme, which is located within the lumen of the endoplasmic reticulum, catalyses the 4-hydroxylation of prolines in -X-Pro-Gly- sequences. The 4-hydroxyproline residues are essential for the formation of the collagen triple helix. The enzyme forms a complex with protein disulfide isomerase and acts not only on procollagen but also on more than 15 other proteins that have collagen-like domains.
History
EC 1.14.11.2 created 1972, modified 1981, modified 1983, modified 2017
Pathway
ec00330  Arginine and proline metabolism
ec01100  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)
NLE: 100593863(P4HA2) 100595647(P4HA1) 100605142(P4HA3)
MCC: 707157(P4HA2) 707927(P4HA1) 718872(P4HA3)
MCF: 101925842(P4HA1) 102119382(P4HA2) 102142383(P4HA3)
CSAB: 103216007(P4HA1) 103244505(P4HA2) 103248029(P4HA3)
RRO: 104659847(P4HA2) 104665124(P4HA3) 104669953(P4HA1)
RBB: 108516851(P4HA1) 108529597(P4HA2) 108537616(P4HA3)
CJC: 100393458(P4HA1) 100406015(P4HA3) 100409805(P4HA2)
MMU: 18451(P4ha1) 18452(P4ha2) 320452(P4ha3)
RNO: 360526(P4ha2) 361612(P4ha3) 64475(P4ha1)
CGE: 100757890(P4ha1) 100760683(P4ha2) 100766769(P4ha3)
NGI: 103736955(P4ha1) 103747022(P4ha2) 103749825(P4ha3)
HGL: 101702867(P4ha1) 101711128(P4ha3) 101724121(P4ha2)
CCAN: 109688245(P4ha1) 109691558(P4ha3) 109696563(P4ha2) 109703038
OCU: 100340270(P4HA1) 100355439(P4HA2) 100356084(P4HA3)
TUP: 102482017(P4HA3) 102488136(P4HA1) 102497816(P4HA2)
CFA: 474672(P4HA2) 479242(P4HA1) 609381(P4HA3)
AML: 100479558(P4HA3) 100480425(P4HA1) 100480972(P4HA2)
UMR: 103663839(P4HA2) 103667815(P4HA1) 103678048(P4HA3)
ORO: 101378303(P4HA3) 101380998(P4HA2) 101385408(P4HA1)
FCA: 101081160(P4HA3) 101094394(P4HA2) 101096846(P4HA1)
PTG: 102954449(P4HA3) 102964926(P4HA2) 102968339(P4HA1)
AJU: 106970713(P4HA2) 106970964(P4HA1) 106978002(P4HA3)
BTA: 414348(P4HA3) 507327(P4HA2) 518288(P4HA1)
BOM: 102274070(P4HA1) 102274248(P4HA2) 102280992(P4HA3)
BIU: 109553629(P4HA1) 109561821(P4HA2) 109569025(P4HA3)
PHD: 102333228(P4HA1) 102335098(P4HA3) 102339818(P4HA2)
CHX: 102179448(P4HA1) 102185942(P4HA3) 102189116(P4HA2)
OAS: 101104856(P4HA3) 101116374(P4HA1) 101123039 101123553(P4HA2)
SSC: 100037299(P4HA1) 100623801(P4HA2) 100624543(P4HA3)
CFR: 102510786(P4HA1) 102516186(P4HA3) 102522559(P4HA2)
CDK: 105090037(P4HA2) 105092471(P4HA1) 105097019(P4HA3)
BACU: 102997313 103004544(P4HA1) 103005873(P4HA2) 103012665(P4HA3)
LVE: 103069084(P4HA1) 103072346(P4HA3) 103089373(P4HA2)
OOR: 101277779(P4HA3) 101287258(P4HA2) 101289964(P4HA1)
ECB: 100063760(P4HA1) 100065260(P4HA3) 100072882(P4HA2)
EPZ: 103563293(P4HA2) 103564586(P4HA3) 103564805(P4HA1)
EAI: 106831684(P4HA3) 106841024(P4HA2) 106842394(P4HA1)
MYB: 102239530 102242052(P4HA2) 102245712(P4HA3) 102255473(P4HA1)
MYD: 102767574(P4HA2) 102771133(P4HA3) 102775235(P4HA1)
HAI: 109371877(P4HA1) 109382575(P4HA2) 109386307(P4HA3)
PALE: 102882040(P4HA3) 102885589(P4HA1) 102892298(P4HA2)
LAV: 100663032(P4HA1) 100668295(P4HA3) 100676749(P4HA2)
MDO: 100010507(P4HA1) 100013447(P4HA3) 100018258(P4HA2)
SHR: 100913891(P4HA1) 100921246(P4HA3) 100922527 100931276(P4HA2)
OAA: 100077226(P4HA1) 100079170(P4HA2)
GGA: 416326(P4HA2) 419057(P4HA3) 423704(P4HA1)
MGP: 100544911(P4HA1) 100547385(P4HA2) 100550944(P4HA3)
CJO: 107308628(P4HA3) 107315698(P4HA1) 107320086(P4HA2)
APLA: 101792390(P4HA2) 101799325(P4HA3) 101802328(P4HA1)
ACYG: 106030561(P4HA1) 106032842(P4HA2)
TGU: 100222421 100223373(P4HA3) 100226743(P4HA2) 100228350(P4HA1)
GFR: 102037596(P4HA2) 102038368(P4HA3) 102040068(P4HA1)
FAB: 101807615(P4HA3) 101808225(P4HA2) 101809059(P4HA1)
PHI: 102110972(P4HA3) 102113563(P4HA2) 102114022(P4HA1)
PMAJ: 107206683(P4HA1) 107210731(P4HA2) 107212878(P4HA3)
CCW: 104695051(P4HA1) 104697984(P4HA2) 104698140(P4HA3)
FPG: 101913115(P4HA2) 101915502(P4HA1) 101921488(P4HA3)
FCH: 102047744(P4HA2) 102052133(P4HA1) 102054422(P4HA3)
CLV: 102092707(P4HA3) 102096249(P4HA1) 102096911(P4HA2)
EGZ: 104128394(P4HA1) 104133056(P4HA3) 104133540(P4HA2)
AAM: 106484158(P4HA3) 106487222(P4HA2) 106498584(P4HA1)
ASN: 102370647(P4HA3) 102373565(P4HA2) 102376142(P4HA1)
AMJ: 102557660(P4HA2) 102569283(P4HA3) 106736856(P4HA1)
PSS: 102449785(P4HA1) 102451448(P4HA2) 102458696(P4HA3)
CMY: 102932371(P4HA1) 102933622(P4HA2) 102935870(P4HA3)
CPIC: 101934564(P4HA3) 101943574(P4HA2) 101946513(P4HA1)
ACS: 100557390(p4ha3) 100565232(p4ha1) 100567447(p4ha2)
PVT: 110073699(P4HA1) 110078142(P4HA3) 110086688(P4HA2)
PBI: 103059803(P4HA1) 103063211(P4HA2) 103066775(P4HA3)
GJA: 107108751(P4HA2) 107124648(P4HA1)
XLA: 108696224 108697480 108707629(p4ha3.L) 108713277 447527(p4ha2.L)
XTR: 100490484(p4ha1) 100492280 100495824(p4ha3) 101734880 493342(p4ha2)
NPR: 108792893(P4HA2) 108799533(P4HA1) 108802239(P4HA3)
DRE: 100003675(p4ha1b) 373132(p4ha2) 562774(p4ha1a) 563281(p4ha3)
TRU: 101064031(p4ha3) 101066030(p4ha1) 101071700 101075755(p4ha2)
LCO: 104917796(p4ha2) 104919527(p4ha1) 104925929(p4ha3) 104938904 109137748
NCC: 104943317 104964567(p4ha1) 104967596(p4ha3)
MZE: 101464180 101464219(p4ha1) 101485561(p4ha3) 101486537(p4ha2)
OLA: 101161986(p4ha3) 101163841(p4ha1) 101166436(p4ha2) 101172363
XMA: 102216605(p4ha1) 102219518(p4ha3) 102220364(p4ha2) 102224850
PRET: 103474134(p4ha3) 103475341(p4ha2) 103476714(p4ha1) 103481891
NFU: 107375942(p4ha1) 107380001(p4ha3) 107386792(p4ha2) 107387232
CSEM: 103378168(p4ha3) 103382259 103387246(p4ha1) 103395274
LCF: 108874730 108876069(p4ha3) 108893921(p4ha2) 108897384(p4ha1)
HCQ: 109510135(p4ha1) 109514510(p4ha3) 109517850(p4ha2) 109518591 109518594
BPEC: 110153980(p4ha2) 110156810(p4ha1) 110163660(p4ha3) 110165450
LCM: 102346232(P4HA2) 102360424(P4HA3) 102363342(P4HA1)
CMK: 103177143(p4ha3) 103181221(p4ha1) 103184118(p4ha2)
SPU: 592929
APLC: 110986832
DME: Dmel_CG11828(CG11828) Dmel_CG1546(PH4alphaSG2) Dmel_CG15539(CG15539) Dmel_CG15864(CG15864) Dmel_CG18231(CG18231) Dmel_CG18749(CG18749) Dmel_CG31013(CG31013) Dmel_CG31014(PH4alphaSG1) Dmel_CG31015(PH4alphaPV) Dmel_CG31016(CG31016) Dmel_CG31017(PH4alphaNE3) Dmel_CG31021(CG31021) Dmel_CG31022(PH4alphaEFB) Dmel_CG31524(CG31524) Dmel_CG32199(CG32199) Dmel_CG32201(CG32201) Dmel_CG34345(CG34345) Dmel_CG4174 Dmel_CG9698 Dmel_CG9720(PH4alphaNE2) Dmel_CG9726(PH4alphaMP) Dmel_CG9728(PH4alphaNE1)
DSI: Dsimw501_GD12338(Dsim_GD12338) Dsimw501_GD14756(Dsim_GD14756) Dsimw501_GD14757(Dsim_GD14757) Dsimw501_GD14758(Dsim_GD14758) Dsimw501_GD16902(Dsim_GD16902) Dsimw501_GD16913(Dsim_GD16913) Dsimw501_GD16980(Dsim_GD16980) Dsimw501_GD16991(Dsim_GD16991) Dsimw501_GD17002(Dsim_GD17002) Dsimw501_GD17024(Dsim_GD17024) Dsimw501_GD17035(Dsim_GD17035) Dsimw501_GD18523(Dsim_GD18523) Dsimw501_GD18524(Dsim_GD18524) Dsimw501_GD21390(Dsim_GD21390) Dsimw501_GD21521(Dsim_GD21521) Dsimw501_GD21523(Dsim_GD21523) Dsimw501_GD21524(Dsim_GD21524) Dsimw501_GD21527(Dsim_GD21527) Dsimw501_GD21535(Dsim_GD21535) Dsimw501_GD21536(Dsim_GD21536) Dsimw501_GD21537(Dsim_GD21537) Dsimw501_GD23422(Dsim_GD23422)
AAG: 5568675
AME: 408862(PH4alphaEFB)
BIM: 100741349
BTER: 100645804
SOC: 105195693
AEC: 105144468
ACEP: 105622522
PBAR: 105431776
HST: 105192505
CFO: 105248930
LHU: 105670346
PGC: 109852101
NVI: 100116138
NVL: 108560083
BMOR: 692683
PRAP: 110992264
DNX: 107173468
ZNE: 110829218
CEL: CELE_F35G2.4(phy-2) CELE_Y47D3B.10(dpy-18)
CBR: CBG06252(Cbr-phy-2) CBG13195(Cbr-dpy-18)
BMY: Bm1_45455
AQU: 100634548
LJA: Lj0g3v0129829.1(Lj0g3v0129829.1) Lj0g3v0186299.1(Lj0g3v0186299.1) Lj0g3v0187809.1(Lj0g3v0187809.1) Lj1g3v0715730.1(Lj1g3v0715730.1) Lj1g3v2251610.1(Lj1g3v2251610.1) Lj2g3v0371560.1(Lj2g3v0371560.1) Lj2g3v1022720.1(Lj2g3v1022720.1) Lj2g3v1648080.1(Lj2g3v1648080.1) Lj2g3v1659670.1(Lj2g3v1659670.1) Lj2g3v1952980.1(Lj2g3v1952980.1) Lj4g3v0771120.1(Lj4g3v0771120.1) Lj4g3v2375000.1(Lj4g3v2375000.1) Lj5g3v2111920.1(Lj5g3v2111920.1) Lj5g3v2302900.1(Lj5g3v2302900.1) Lj6g3v1915970.1(Lj6g3v1915970.1)
POP: 7469094
DOSA: Os01t0174500-01(Os01g0174500) Os03t0166100-00(Os03g0166100) Os03t0166200-00(Os03g0166200) Os03t0761900-01(Os03g0761900) Os03t0803500-01(Os03g0803500) Os04t0346000-01(Os04g0346000) Os05t0489100-01(Os05g0489100) Os07t0194500-01(Os07g0194500) Os10t0413500-01(Os10g0413500) Os10t0497800-01(Os10g0497800)
ATS: 109739645(LOC109739645) 109746572(LOC109746572) 109757842(LOC109757842) 109758919(LOC109758919) 109759038(LOC109759038) 109763191(LOC109763191) 109765848(LOC109765848) 109765968(LOC109765968) 109766734(LOC109766734)
ZMA: 100193348 100193935(pco071536) 100272381 100274058 100282945(pco107217) 100283657(cl32377_1(260)) 100284871(pco093706) 100303994 100383727
NTE: NEUTE1DRAFT122243(NEUTE1DRAFT_122243)
CMT: CCM_06081
ANI: AN2851.2
ANG: ANI_1_2732024(An02g07870)
PTE: PTT_06584
DFA: DFA_03579
TAN: TA04445
SMIN: v1.2.000155.t2(symbB.v1.2.000155.t2) v1.2.001198.t1(symbB.v1.2.001198.t1) v1.2.007318.t1(symbB.v1.2.007318.t1) v1.2.013112.t3(symbB.v1.2.013112.t3) v1.2.013112.t4(symbB.v1.2.013112.t4) v1.2.017644.t1(symbB.v1.2.017644.t1) v1.2.021695.t1(symbB.v1.2.021695.t1) v1.2.038749.t1(symbB.v1.2.038749.t1) v1.2.038749.t2(symbB.v1.2.038749.t2)
XCC: XCC3429
XCB: XC_0735
XCP: XCR_3769
XCV: XCV0752
XAX: XACM_0696
XAC: XAC0691
XPH: XppCFBP6546_08120(XppCFBP6546P_08120)
PSD: DSC_10945
LEZ: GLE_1977
PSM: PSM_A2325
AMAL: I607_05385
AMAE: I876_05675
AMAO: I634_05700
AMAD: I636_05620
AMAI: I635_05585
AMAG: I533_05275
AAUS: EP12_03625
SAGA: M5M_16075
TKM: TK90_2795
RSO: RSc2814
RSE: F504_2741
RPI: Rpic_3059
REH: H16_A3244(phy)
CNC: CNE_1c31800(phy)
RME: Rmet_3098
BDL: AK34_3751
BGO: BM43_6215
BPH: Bphy_5329
BFN: OI25_6958
POL: Bpro_3712
AAV: Aave_1171
AJS: Ajs_3293
AAA: Acav_1184
ACRA: BSY15_2583
DAC: Daci_5284
CTES: O987_05330
RTA: Rta_28970
HYB: Q5W_15775
CBAA: SRAA_1518
CBAB: SMCB_0491
MPT: Mpe_A2091
CFU: CFU_2670
LCH: Lcho_2651
RGE: RGE_13160
BBT: BBta_1313
HNE: HNE_1861
NAR: Saro_1382
SAL: Sala_1522
SPHP: LH20_07745
SMAZ: LH19_12780
SWI: Swit_2573
SPHD: HY78_12650
AFR: AFE_1210
BAN: BA_4459
BAR: GBAA_4459
BAT: BAS4138
BAI: BAA_4476
BANT: A16_44550
BANR: A16R_45100
BANS: BAPAT_4277
BANV: DJ46_3142
BCE: BC4233
BCA: BCE_4314
BCQ: BCQ_4020
BCX: BCA_4345
BNC: BCN_4148
BCF: bcf_21065
BCER: BCK_13995
BTL: BALH_3835
BTT: HD73_4540
BTHI: BTK_22365
BTM: MC28_3528
BTI: BTG_28220
BTW: BF38_5402
BWW: bwei_0700(phy)
BMYO: BG05_1782
BMQ: BMQ_2841
BMD: BMD_2875
BMEG: BG04_5381
BACO: OXB_0125
BEO: BEH_13330
SIV: SSIL_1645
JEO: JMA_36400
SYW: SYNW2355
SYNR: KR49_08085
SYND: KR52_03445
 » show all
Taxonomy
Reference
1
  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
2
  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.
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.
Reference
4  [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.
Reference
5  [PMID:7947811]
  Authors
John DC, Bulleid NJ
  Title
Prolyl 4-hydroxylase: defective assembly of alpha-subunit mutants indicates that  assembled alpha-subunits are intramolecularly disulfide bonded.
  Journal
Biochemistry. 33 (1994) 14018-25.
Reference
6  [PMID:7730375]
  Authors
Lamberg A, Pihlajaniemi T, Kivirikko KI
  Title
Site-directed mutagenesis of the alpha subunit of human prolyl 4-hydroxylase. Identification of three histidine residues critical for catalytic activity.
  Journal
J. Biol. Chem. 270 (1995) 9926-31.
Reference
7  [PMID:9135134]
  Authors
Myllyharju J, Kivirikko KI
  Title
Characterization of the iron- and 2-oxoglutarate-binding sites of human prolyl 4-hydroxylase.
  Journal
EMBO. J. 16 (1997) 1173-80.
Reference
8  [PMID:9524356]
  Authors
Kivirikko KI, Myllyharju J
  Title
Prolyl 4-hydroxylases and their protein disulfide isomerase subunit.
  Journal
Matrix. Biol. 16 (1998) 357-68.
Other DBs
ExplorEnz - The Enzyme Database: 1.14.11.2
IUBMB Enzyme Nomenclature: 1.14.11.2
ExPASy - ENZYME nomenclature database: 1.14.11.2
BRENDA, the Enzyme Database: 1.14.11.2
CAS: 9028-06-2

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