KEGG   ENZYME: 1.14.11.8
Entry
EC 1.14.11.8                Enzyme                                 
Name
trimethyllysine dioxygenase;
trimethyllysine alpha-ketoglutarate dioxygenase;
TML-alpha-ketoglutarate dioxygenase;
TML hydroxylase;
6-N,6-N,6-N-trimethyl-L-lysine,2-oxoglutarate:oxygen oxidoreductase (3-hydroxylating)
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
Sysname
N6,N6,N6-trimethyl-L-lysine,2-oxoglutarate:oxygen oxidoreductase (3-hydroxylating)
Reaction(IUBMB)
N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 = (3S)-3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 [RN:R03451]
Reaction(KEGG)
R03451
Substrate
N6,N6,N6-trimethyl-L-lysine [CPD:C03793];
2-oxoglutarate [CPD:C00026];
O2 [CPD:C00007]
Product
(3S)-3-hydroxy-N6,N6,N6-trimethyl-L-lysine [CPD:C01259];
succinate [CPD:C00042];
CO2 [CPD:C00011]
Comment
Requires Fe2+ and ascorbate.
History
EC 1.14.11.8 created 1983
Pathway
ec00310  Lysine degradation
ec01100  Metabolic pathways
Orthology
K00474  trimethyllysine dioxygenase
Genes
HSA55217(TMLHE)
PTR107966584(TMLHE)
PPS100989743(TMLHE)
GGO101130045(TMLHE)
PON100459640(TMLHE)
NLE100595389(TMLHE)
HMH116812134(TMLHE)
MCC703711(TMLHE)
MCF102133795(TMLHE)
MTHB126945477
MNI105467979(TMLHE)
CSAB103232863(TMLHE)
CATY105575881(TMLHE)
PANU101008402(TMLHE)
TGE112616159(TMLHE)
MLEU105541575(TMLHE)
RRO104662412(TMLHE)
RBB108521721(TMLHE)
TFN117077204(TMLHE)
PTEH111527386(TMLHE)
CANG105501735(TMLHE)
CJC100396173(TMLHE)
SBQ101039644(TMLHE)
CIMI108294365(TMLHE)
CSYR103252149(TMLHE)
MMUR105871572(TMLHE)
LCAT123628307(TMLHE)
PCOQ105824650(TMLHE)
MMU192289(Tmlhe)
MCAL110287594(Tmlhe)
MPAH110314603(Tmlhe)
RNO170898(Tmlhe)
MCOC116074227(Tmlhe)
ANU117695337(Tmlhe)
MUN110543623
CGE100757720(Tmlhe)
MAUA101827119(Tmlhe)
PROB127218406(Tmlhe)
PLEU114706214(Tmlhe)
MORG121436633(Tmlhe) 121437077
MFOT126490207
AAMP119805037(Tmlhe)
NGI103741300(Tmlhe)
HGL101700950(Tmlhe)
CPOC100730686(Tmlhe)
CCAN109699598(Tmlhe)
DORD106000175(Tmlhe)
DSP122097068(Tmlhe)
PLOP125343438(Tmlhe)
NCAR124972603
MMMA107161016(Tmlhe)
OCU100338782
OPI101518115(TMLHE)
GVR103592835(TMLHE)
CFA481090(TMLHE)
CLUD112668327(TMLHE)
VVP112912243(TMLHE)
VLG121482762(TMLHE)
NPO129518223(TMLHE)
AML100484567(TMLHE)
UMR103659283(TMLHE)
UAH113248335(TMLHE)
UAR123786646(TMLHE)
ELK111139188
LLV125091762
MPUF101681440(TMLHE)
MNP132006835(TMLHE)
MLK131820887(TMLHE)
NVS122896865(TMLHE)
ORO101362600(TMLHE)
EJU114201933(TMLHE)
ZCA113931093(TMLHE)
MLX118014281(TMLHE)
NSU110573998(TMLHE)
LWW102745154(TMLHE)
FCA101094166(TMLHE)
PYU121024839(TMLHE)
PCOO112871174(TMLHE)
PBG122477451(TMLHE)
LRUF124527627
PTG102953719(TMLHE)
PPAD109260781(TMLHE)
PUC125931438
AJU106987211
HHV120227179(TMLHE)
BTA535630(TMLHE)
BOM102265906(TMLHE)
BIU109554807
BBUB102391190(TMLHE)
CHX102188121(TMLHE)
OAS101106308(TMLHE)
BTAX128070410(TMLHE)
ODA120881585(TMLHE)
CCAD122435043(TMLHE)
MBEZ129564373(TMLHE)
SSC100515702(TMLHE)
CFR102524405(TMLHE)
CBAI105078533(TMLHE)
CDK105089418(TMLHE)
VPC102534481(TMLHE)
BACU103017558(TMLHE)
LVE103082632(TMLHE)
OOR101287109(TMLHE)
DLE111170736(TMLHE)
PCAD102991510(TMLHE)
PSIU116747673(TMLHE)
NASI112412549(TMLHE)
ECB100062170(TMLHE)
EPZ103541356(TMLHE)
EAI106827068(TMLHE)
HAI109373621(TMLHE)
RFQ117030366(TMLHE)
PALE102877948(TMLHE)
RAY107507229(TMLHE)
MJV108409659(TMLHE)
LAV100658531(TMLHE)
TMU101360038
DNM101416979(TMLHE)
MDO100024134(TMLHE)
GAS123253783(TMLHE)
SHR100920162(TMLHE)
AFZ127542505
PCW110200320(TMLHE)
OAA100084207(TMLHE)
GGA422296(TMLHE)
PCOC116229710(TMLHE)
MGP100544092(TMLHE)
CJO107312861(TMLHE)
TPAI128075948(TMLHE)
LMUT125699788(TMLHE)
NMEL110403216(TMLHE)
APLA101794183 119712983
ACYG106047518(TMLHE)
CATA118245484(TMLHE)
AFUL116494449(TMLHE)
TGU115495259(TMLHE)
LSR110477977(TMLHE)
SCAN103816317(TMLHE)
PMOA120512643(TMLHE)
OTC121346431(TMLHE)
PRUF121362837(TMLHE)
GFR102041991(TMLHE)
FAB101816366(TMLHE)
OMA130252998(TMLHE)
PHI102099827(TMLHE)
PMAJ107204076(TMLHE)
CCAE111929269(TMLHE)
CCW104691701(TMLHE)
CBRC103622035(TMLHE)
ETL114062097(TMLHE)
ZAB102063262(TMLHE)
ACHL103797623(TMLHE)
SVG106862176(TMLHE)
MMEA130578712(TMLHE)
HRT120761976(TMLHE)
FPG101920173(TMLHE)
FCH102049828(TMLHE)
CLV102085186(TMLHE)
EGZ104129538(TMLHE)
NNI104021103(TMLHE)
PCRI104036531(TMLHE)
PLET104617113(TMLHE)
EHS104513751(TMLHE)
PCAO104044944(TMLHE)
ACUN113480004(TMLHE)
TALA104365867(TMLHE)
PADL103925893(TMLHE)
AFOR103894084(TMLHE)
ACHC115333627(TMLHE)
HALD104322286(TMLHE)
HLE104832749(TMLHE)
AGEN126050066
GCL127019448
CCRI104166741(TMLHE)
CSTI104554319(TMLHE)
CMAC104483345(TMLHE)
MUI104539364(TMLHE)
BREG104639213(TMLHE)
FGA104081790(TMLHE)
GSTE104256551(TMLHE)
MNB103770708(TMLHE)
OHA104333421(TMLHE)
NNT104402286(TMLHE)
SHAB115612711(TMLHE)
DPUB104307222(TMLHE)
PGUU104459058(TMLHE)
ACAR104530547(TMLHE)
CPEA104393949(TMLHE)
AVIT104277008(TMLHE)
CVF104282698(TMLHE)
CUCA104060481(TMLHE)
TEO104371769(TMLHE)
BRHI104492488(TMLHE)
AAM106489870(TMLHE)
AROW112978565(TMLHE)
NPD112947039(TMLHE)
TGT104574261
DNE112982654(TMLHE)
SCAM104154010(TMLHE)
ASN102383970(TMLHE)
AMJ102558694(TMLHE)
GGN109300675(TMLHE)
PSS102450043(TMLHE)
CMY102943917(TMLHE)
CCAY125642236(TMLHE)
DCC119861742(TMLHE)
CPIC101937011(TMLHE)
TST117883036(TMLHE)
CABI116820082(TMLHE)
MRV120371676(TMLHE)
ACS100568254(tmlhe)
ASAO132762980(TMLHE)
PVT110084930(TMLHE)
SUND121936961(TMLHE)
PBI103053306(TMLHE)
PMUR107295409(TMLHE)
CTIG120302672(TMLHE)
TSR106544209(TMLHE)
PGUT117669526(TMLHE)
APRI131205188(TMLHE)
PTEX113437829(TMLHE)
NSS113416604(TMLHE)
VKO123022046(TMLHE)
PMUA114589071(TMLHE)
PRAF128406349(TMLHE)
ZVI118078977(TMLHE)
HCG128335699(TMLHE)
GJA107118207(TMLHE)
STOW125443119(TMLHE)
EMC129341368(TMLHE)
XLA100189568(tmlhe.L) 399066(tmlhe.S)
XTR100124877(tmlhe)
NPR108797003(TMLHE)
RTEM120913582 120924999
BBUF121009345(TMLHE)
BGAR122946378(TMLHE)
MUO115474115(TMLHE)
GSH117361647(TMLHE)
DRE555271(tmlhe)
SRX107715863 107742426
SANH107661707 107703088
SGH107562156 107576017 107581969
CCAR109046117(tmlhe) 109046121
CAUA113038284 113063911
CGIB127942191 127986452
PTET122326138
LROH127152780(tmlhe)
OMC131529134(tmlhe)
PPRM120473675(tmlhe) 120473684
RKG130096836(tmlhe)
MAMB125253036(tmlhe)
CIDE127504141
MASI127429350 127430672
TROS130570596(tmlhe)
TDW130408339(tmlhe)
MANU129423454(tmlhe)
IPU108278764
IFU128622084(tmlhe)
PHYP113541629(tmlhe)
SMEO124397756(tmlhe)
TFD113647598(tmlhe)
TVC132860093(tmlhe)
AMEX103033955(tmlhe)
CMAO118820021(tmlhe)
EEE113571246(tmlhe)
CHAR105889620(tmlhe)
TRU101077347(tmlhe)
TFS130537148(tmlhe)
TNGGSTEN00020373G001
LCO104920012(tmlhe)
NCC104948915
TBEN117499775(tmlhe)
CGOB115019442(tmlhe)
PGEO117458743(tmlhe)
GACU117552969(tmlhe)
ELY117263706(tmlhe)
EFO125899024(tmlhe)
PLEP121952844(tmlhe)
SLUC116058300(tmlhe)
ECRA117955076(tmlhe)
ESP116699963(tmlhe)
PFLV114566948(tmlhe)
GAT120822672(tmlhe)
PPUG119215839(tmlhe)
AFB129113171(tmlhe)
CLUM117743129(tmlhe)
MSAM119884728 119895941
SCHU122888352(tmlhe)
CUD121518417(tmlhe)
ALAT119030953(tmlhe)
MZE101473435(tmlhe)
ONL100708911(tmlhe)
OAU116329147(tmlhe)
OLA101160186(tmlhe)
OML112142999(tmlhe)
XMA102235647(tmlhe)
XCO114152696(tmlhe)
XHE116728961(tmlhe)
PRET103461226 103461227
PFOR103133909(tmlhe)
PLAI106949247(tmlhe)
PMEI106922780(tmlhe)
GAF122844347(tmlhe)
CVG107082568(tmlhe)
CTUL119788064(tmlhe)
GMU124875999(tmlhe)
NFU107386621(tmlhe)
KMR108233816(tmlhe)
ALIM106523113(tmlhe)
NWH119420276(tmlhe)
AOCE111571982(tmlhe)
MCEP125021751(tmlhe)
CSEM103394733(tmlhe)
POV109628321(tmlhe)
SSEN122779677(tmlhe)
HHIP117774644(tmlhe)
HSP118121692(tmlhe)
LCF108893980
SDU111222374(tmlhe)
SLAL111654852 111663821(tmlhe)
XGL120802132(tmlhe)
HCQ109508849(tmlhe)
SSCV125982192
SBIA133491724(tmlhe)
PEE133415929(tmlhe)
PTAO133466795(tmlhe)
BPEC110165115(tmlhe)
MALB109960422(tmlhe)
BSPL114869040(tmlhe)
SJO128371423(tmlhe)
SASA106563395 106603194(TMLH)
STRU115154641 115204242(tmlhe)
OTW112228784 112231363
OMY110509935 110533947
OGO123993975 124006980
ONE115101297(tmlhe) 115135673
OKI109873436 109873804(tmlhe)
OKE118385155 118390324
SALP111950794 111964143
SNH120021565 120057220
CCLU121541803 121580329
ELS105024591(tmlhe)
SFM108919972(tmlhe)
PKI111860885(tmlhe)
AANG118236756(tmlhe)
LOC102696971(tmlhe)
PSPA121295909(tmlhe)
ARUT117430452(tmlhe)
PSEX120537106(tmlhe)
LCM102359408(TMLHE)
CMK103177255(tmlhe)
RTP109937452(tmlhe)
CPLA122557186(tmlhe)
HOC132820493(tmlhe)
LERI129702189(tmlhe)
PMRN116957152(TMLHE)
BFO118427387 118431537
BBEL109477774
CIN100185738
SCLV120333428 120345893
SPU584681
AJC117121494
SKO100375730
DER6553722
DSIDsimw501_GD20068(Dsim_GD20068)
DYADyak_GE25077
DSR110188200
DPO4801861
DPE6587962
DMN108157067
DWI6649504
DMODmoj_GI10544
DAZ108621783
DNV108660032
DHE111595978
DVI6630396
MDE101892707 101901622 101901706
SCAC106089898
LCQ111680683 111682310
LSQ119607815 119610660
ACOZ120948709
AAG5572349
AALB109409986 115260850
CQUCpipJ_CPIJ012189
CPII120420561
NVL108557604
PPYR116179335
OTU111420815
BMOR101743938
BMAN114241819
DPLKGM_205173
PMAC106715682
PPOT106103355
PXU106113513
PRAP110994512
ZCE119833388 119833659
LSIN126975218
HAW110377408
TNL113506445 113507507
PXY105382967
API100168280
DNX107167220
AGS114123610 114129344
RMD113553251
BTAB109034567
NLU111062665
HVI124360493
PHUPhum_PHUM613090
FOC113217740
TPAL117650056
ZNE110837782
CSEC111873736
SGRE126298275
FCD110856262
DPXDAPPUDRAFT_306710
PVM113818300
PJA122248572
HAME121853459
CQD128704403
PTRU123503231
ESN126980354
HAZT108673074
LSM121115036
TCF131885438
PPOI119096555 119110200
DSV119455633
RSAN119393929
LPOL106459873
PMEO129597724
NAINECAME_06933
HROHELRODRAFT_161402
LGILOTGIDRAFT_216511
PVUL126808807 126827778
PCAN112555638
BGT106076854
GAE121371387 121390808
HRF124111148
HRJ124285314
CRG105348070
CVN111132862
CANU128181299
OED125658898
MYI110446010
PMAX117342502
LAK106172265
NVE5501991 5506894 5511683
EPA110240651 110242183 110250187
ATEN116304915 116307327
ADF107354987
AMIL114958634
PDAM113664613
SPIS111323987
HSY130614596
TADTRIADDRAFT_61285
AQU100633127 105312230
MISMICPUN_102113
AGOAGOS_AEL035W
ERCEcym_1338
KLAKLLA0_A10945g
KMXKLMA_10537
LTHKLTH0H13574g
PPAPAS_chr1-1_0129
DHADEHA2F13046g
PICPICST_46425(TML1)
PGUPGUG_00921
SPAASPAPADRAFT_138818
LELPVL30_004948
CALCAALFM_C502920WA(CaO19.4316)
CTPCTRG_05994
COTCORT_0F01450
CDUCD36_52680
CTENCANTEDRAFT_131558
YLIYALI0C10604g
CLUCLUG_01134
CLUSA9F13_09g01947
CAURCJI96_0003285
NCRNCU03802(cbs-1)
NTENEUTE1DRAFT76877(NEUTE1DRAFT_76877)
PANPODANSg9037
CTHRCTHT_0067480
MGRMGG_07454
PPEIPpBr36_02363
PGRIPgNI_02415
TMNUCRPA7_3046
SSCKSPSK_02400
FGRFGSG_06105
FPUFPSE_04261
FPOAFPOAC1_009080
FVNFVRRES_09700
FVRFVEG_04736
FOXFOXG_07816
NHENECHADRAFT_49296
FFCNCS54_01155100
FMUJ7337_006771
TRETRIREDRAFT_60232
TRRM419DRAFT_7919
MAWMAC_08951
MAJMAA_08416
PCHMVFPPC_13937
CMTCCM_07727
AMUSLMH87_002592
PLJVFPFJ_08089
PTKZJDV02_005009
VALVDBG_00078
VDAVDAG_08368
CFJCFIO01_13670
CHIGCH63R_04125
SAPOSAPIO_CDS10392
ELAUCREL1_4731
PFYPFICI_08323
SSLSS1G_07291
BFUBCIN_10g02100
MBEMBM_01235
PSCOLY89DRAFT_63859 LY89DRAFT_681525 LY89DRAFT_747795
GLZGLAREA_01784
AFMAFUA_1G06180
ACTACLA_028230
NFINFIA_018530
AORAO090001000433
ANGAn18g05220
AFVAFLA_012547
ALUCAKAW2_31099A
ACHEACHE_11086S
APUUAPUU_60482S
PCSN7525_000248
PDPPDIP_69230
POUPOX_e06660
TMFEYB26_000450
TRGTRUGW13939_04417
CIMCIMG_03536
CPWCPC735_007140
UREUREG_07329
PBLPAAG_01927
PBNPADG_00600
ABEARB_05432
TVETRV_03837
AJEHCAG_00068
BGHBDBG_06330
PNOSNOG_08253
PTEPTT_08702
BZECOCCADRAFT_41337
BSCCOCSADRAFT_163529
BORCOCMIDRAFT_38194
AALTCC77DRAFT_1078322
ARABEKO05_0004820
ZTRMYCGRDRAFT_103060
PFJMYCFIDRAFT_185248
FFUCLAFUR5_10652
CBETCB0940_06400
BCOMBAUCODRAFT_48091
TMLGSTUM_00001459001
CNECNF02980
CNBCNBF1730
CNGCNAG_05675
CGICGB_F2360C
TMSTREMEDRAFT_69890
TASAA1Q1_04465
CCACCcaHIS019_0405460(CcaverHIS019_0405460)
TVSTRAVEDRAFT_150399
DSQDICSQDRAFT_178141
PCOPHACADRAFT_252822
SHSSTEHIDRAFT_90972
HIRHETIRDRAFT_473978
PSQPUNSTDRAFT_66109
ADLAURDEDRAFT_112684
FMEFOMMEDRAFT_152004
GTRGLOTRDRAFT_46394
LBCLACBIDRAFT_249619
CCICC1G_06306
PCUBJR316_0006222
MPRMPER_12193
MRRMoror_4598
MOREE1B28_011493
SCMSCHCO_02688622(SCHCODRAFT_02688622)
CPUTCONPUDRAFT_151231
WSEWALSEDRAFT_59031
WICJ056_004465
UMAUMAG_11458
PFPPFL1_00677
SGRAEX895_003396
PGRPGTG_19005
PTRCPtA15_2A143
PSTRPst134EA_004753
MLRMELLADRAFT_70609
MBRMONBRDRAFT_23751
SREPTSG_01787
SMINv1.2.019441.t1(symbB.v1.2.019441.t1)
FCYFRACYDRAFT_262191(TMLD)
PIFPITG_04618
PSOJPHYSODRAFT_566756
SPARSPRG_03676 SPRG_18381 SPRG_21026
EHXEMIHUDRAFT_451329
GTTGUITHDRAFT_155346
NGRNAEGRDRAFT_69583
SPLSpea_2204
SWDSwoo_2344
THTE2K93_06085
TSNW908_07190
THINCRN91_03065
 » show all
Reference
1  [PMID:627563]
  Authors
Hulse JD, Ellis SR, Henderson LM.
  Title
Carnitine biosynthesis. beta-Hydroxylation of trimethyllysine by an alpha-ketoglutarate-dependent mitochondrial dioxygenase.
  Journal
J Biol Chem 253:1654-9 (1978)
Reference
2  [PMID:27730239]
  Authors
Al Temimi AH, Pieters BJ, Reddy YV, White PB, Mecinovic J
  Title
Substrate scope for trimethyllysine hydroxylase catalysis.
  Journal
Chem Commun (Camb) 52:12849-12852 (2016)
DOI:10.1039/c6cc07845a
Reference
3  [PMID:27965989]
  Authors
Lesniak RK, Markolovic S, Tars K, Schofield CJ
  Title
Human carnitine biosynthesis proceeds via (2S,3S)-3-hydroxy-N(epsilon)-trimethyllysine.
  Journal
Chem Commun (Camb) 53:440-442 (2016)
DOI:10.1039/c6cc08381a
  Sequence
[hsa:55217]
Reference
4  [PMID:28045275]
  Authors
Reddy YV, Al Temimi AH, White PB, Mecinovic J
  Title
Evidence That Trimethyllysine Hydroxylase Catalyzes the Formation of (2S,3S)-3-Hydroxy-N(epsilon)-trimethyllysine.
  Journal
Org Lett 19:400-403 (2017)
DOI:10.1021/acs.orglett.6b03608
  Sequence
[hsa:55217]
Other DBs
ExplorEnz - The Enzyme Database: 1.14.11.8
IUBMB Enzyme Nomenclature: 1.14.11.8
ExPASy - ENZYME nomenclature database: 1.14.11.8
BRENDA, the Enzyme Database: 1.14.11.8
CAS: 74622-49-4

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