Entry
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
BRITE hierarchy
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)
Substrate
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
Orthology
K00474 trimethyllysine dioxygenase
Genes
MORG : 121436633(Tmlhe) 121437077
APLA : 101794183 119712983
XLA : 100189568(tmlhe.L) 399066(tmlhe.S)
RTEM : 120913582 120924999
SANH : 107661707 107703088
SGH : 107562156 107576017 107581969
CCAR : 109046117(tmlhe) 109046121
CAUA : 113038284 113063911
CGIB : 127942191 127986452
PPRM : 120473675(tmlhe) 120473684
MASI : 127429350 127430672
MSAM : 119884728 119895941
ASTR : 137606191 137606761
PRET : 103461226 103461227
SLAL : 111654852 111663821(tmlhe)
SASA : 106563395 106603194(TMLH)
STRU : 115154641 115204242(tmlhe)
ONE : 115101297(tmlhe) 115135673
OKI : 109873436 109873804(tmlhe)
SALP : 111950794 111964143
CCLU : 121541803 121580329
SCLV : 120333428 120345893
DSI : Dsimw501_GD20068(Dsim_GD20068)
AALB : 109409986 115260850
BPHI : 137928847 137928853
PPOI : 119096555 119110200
PVUL : 126808807 126827778
AIRR : 138307369 138307960 138318571
LLON : 135486887 135494642
NVE : 5501991 5506894 5511683
EPA : 110240651 110242183 110250187
ATEN : 116304915 116307327
LBG : 92210533(LODBEIA_P53370)
CAL : CAALFM_C502920WA(CaO19.4316)
CTEN : 18249538(PSN45_000864)
YLI : 2909477(YALI2_C00990g)
NTE : NEUTE1DRAFT76877(NEUTE1DRAFT_76877)
SMP : 90947722(SMAC4_12829)
TATV : 25782661(TrAtP1_011354)
TASP : 36613434(TrAFT101_008748)
MAW : 19253262(J3458_020840)
PLJ : 28890212(PLICBS_009552)
CDET : 87941158(CDEST_04655)
PSCO : LY89DRAFT_63859 LY89DRAFT_681525 LY89DRAFT_747795
CPW : 90964967(D8B26_003071)
PTRR : 6346816(PtrM4_146950)
KMG : 30161013(I203_105383)
KNE : 92184347(IAR55_007089)
CCAC : CcaHIS019_0405460(CcaverHIS019_0405460)
SCM : SCHCO_02688622(SCHCODRAFT_02688622)
FCY : FRACYDRAFT_262191(TMLD)
SPAR : SPRG_03676 SPRG_18381 SPRG_21026
» show all
Taxonomy
Reference
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
Authors
Al Temimi AH, Pieters BJ, Reddy YV, White PB, Mecinovic J
Title
Substrate scope for trimethyllysine hydroxylase catalysis.
Journal
Reference
Authors
Lesniak RK, Markolovic S, Tars K, Schofield CJ
Title
Human carnitine biosynthesis proceeds via (2S,3S)-3-hydroxy-N(epsilon)-trimethyllysine.
Journal
Sequence
Reference
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
Sequence
Other DBs
ExPASy - ENZYME nomenclature database: 1.14.11.8
LinkDB
All DBs