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Protein

Trimethyllysine dioxygenase, mitochondrial

Gene

Tmlhe

Organism
Rattus norvegicus (Rat)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML) (PubMed:11431483).1 Publication

Catalytic activityi

N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 = 3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2.

Cofactori

Protein has several cofactor binding sites:
  • Fe2+By similarityNote: Binds 1 Fe2+ ion per subunit.By similarity
  • L-ascorbate

Pathwayi: carnitine biosynthesis

This protein is involved in the pathway carnitine biosynthesis, which is part of Amine and polyamine biosynthesis.
View all proteins of this organism that are known to be involved in the pathway carnitine biosynthesis and in Amine and polyamine biosynthesis.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi242Iron; catalyticBy similarity1
Metal bindingi244Iron; catalyticBy similarity1
Metal bindingi389Iron; catalyticBy similarity1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionDioxygenase, Oxidoreductase
Biological processCarnitine biosynthesis
LigandIron, Metal-binding

Enzyme and pathway databases

BioCyciMetaCyc:MONOMER-14429
BRENDAi1.14.11.8 5301
ReactomeiR-RNO-71262 Carnitine synthesis
UniPathwayiUPA00118

Names & Taxonomyi

Protein namesi
Recommended name:
Trimethyllysine dioxygenase, mitochondrial (EC:1.14.11.8)
Alternative name(s):
Epsilon-trimethyllysine 2-oxoglutarate dioxygenase
TML hydroxylase
TML-alpha-ketoglutarate dioxygenase
Short name:
TML dioxygenase
Short name:
TMLD
Gene namesi
Name:Tmlhe
Synonyms:Tmlh
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Chromosome 20

Organism-specific databases

RGDi620629 Tmlhe

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 15MitochondrionBy similarityAdd BLAST15
ChainiPRO_000000279716 – 421Trimethyllysine dioxygenase, mitochondrialAdd BLAST406

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei179N6-acetyllysineBy similarity1
Modified residuei236N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

PaxDbiQ91ZW6
PRIDEiQ91ZW6

PTM databases

iPTMnetiQ91ZW6
PhosphoSitePlusiQ91ZW6

Expressioni

Gene expression databases

BgeeiENSRNOG00000000729
GenevisibleiQ91ZW6 RN

Interactioni

Subunit structurei

Homodimer.1 Publication

Protein-protein interaction databases

IntActiQ91ZW6, 1 interactor
STRINGi10116.ENSRNOP00000044070

Structurei

3D structure databases

ProteinModelPortaliQ91ZW6
SMRiQ91ZW6
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the gamma-BBH/TMLD family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG3889 Eukaryota
COG2175 LUCA
GeneTreeiENSGT00530000063582
HOGENOMiHOG000210004
HOVERGENiHBG035650
InParanoidiQ91ZW6
KOiK00474
OMAiKRRMCGA
OrthoDBiEOG091G062L
PhylomeDBiQ91ZW6
TreeFamiTF313805

Family and domain databases

Gene3Di3.30.2020.30, 1 hit
InterProiView protein in InterPro
IPR038492 GBBH-like_N_sf
IPR010376 GBH-like_N
IPR003819 TauD/TfdA-like
IPR012776 Trimethyllysine_dOase
PfamiView protein in Pfam
PF06155 DUF971, 1 hit
PF02668 TauD, 1 hit
TIGRFAMsiTIGR02410 carnitine_TMLD, 1 hit

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q91ZW6-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MWYHKLLHQQ SRLQNLMKRG DIAHGLRLSG FKSLFPFSLH WCHTASKSVN
60 70 80 90 100
CTWHQHEDHL ELQYASTVMR FDYVWLRDHC RSASCYNSKT HQRSLDTASV
110 120 130 140 150
DLCIKPKTIR LDESTLFFTW PDGHVTRYDL DWLVKNSYEG QKQEVIQPRV
160 170 180 190 200
LWNAKLYQDA QLPSVDFQGF LETKEGLKKF LQNFLLYGIA FVENVPPTQE
210 220 230 240 250
HTEKLARRVS LIRETIYGRM WYFTSDFSRG DTAYTKLALD RHTDTTYFQE
260 270 280 290 300
PCGIQVFHCL KHEGTGGRTL LVDGFYAAQQ VLQRAPEEFD LLSQVPLKHE
310 320 330 340 350
YIENVGQCHN HMIGVGPILN IYPWNKELYL IRYNNYDRAV INTVPYDVVR
360 370 380 390 400
RWYAAHRTLT TELRRPENEL WVKLKPGKVL FIDNWRVLHG RESFTGYRQL
410 420
CGCYLTRDDV LNTARILGLH A
Length:421
Mass (Da):49,568
Last modified:November 28, 2006 - v2
Checksum:i487AB5FCA0D02D25
GO
Isoform 2 (identifier: Q91ZW6-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     62-62: L → LGTKPRALRLLEL

Note: No experimental confirmation available.
Show »
Length:433
Mass (Da):50,916
Checksum:iF363DFA37363D692
GO

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_02158062L → LGTKPRALRLLEL in isoform 2. 1 Publication1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
BC085923 mRNA Translation: AAH85923.1
AF374406 mRNA Translation: AAL01252.1
RefSeqiNP_596878.2, NM_133387.2 [Q91ZW6-2]
XP_006255917.1, XM_006255855.1 [Q91ZW6-1]
UniGeneiRn.3607

Genome annotation databases

EnsembliENSRNOT00000049573; ENSRNOP00000044070; ENSRNOG00000000729 [Q91ZW6-2]
ENSRNOT00000076655; ENSRNOP00000068322; ENSRNOG00000000729 [Q91ZW6-1]
ENSRNOT00000076901; ENSRNOP00000068031; ENSRNOG00000000729 [Q91ZW6-2]
GeneIDi170898
KEGGirno:170898
UCSCiRGD:620629 rat [Q91ZW6-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiTMLH_RAT
AccessioniPrimary (citable) accession number: Q91ZW6
Secondary accession number(s): Q5U2P7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 20, 2002
Last sequence update: November 28, 2006
Last modified: March 28, 2018
This is version 118 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health