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Protein

Trimethyllysine dioxygenase, mitochondrial

Gene

Tmlhe

Organism
Rattus norvegicus (Rat)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

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

Catalytic activityi

N6,N6,N(6)-trimethyl-L-lysine + 2-oxoglutarate + O2 = 3-hydroxy-N6,N6,N(6)-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

Complete GO annotation...

Keywords - Molecular functioni

Dioxygenase, Oxidoreductase

Keywords - Biological processi

Carnitine biosynthesis

Keywords - Ligandi

Iron, 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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Chromosome 20

Organism-specific databases

RGDi620629. Tmlhe.

Subcellular locationi

GO - Cellular componenti

  • mitochondrial matrix Source: UniProtKB-SubCell
  • mitochondrion Source: RGD
Complete GO annotation...

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.
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.
OMAiIEINEFW.
OrthoDBiEOG091G062L.
PhylomeDBiQ91ZW6.
TreeFamiTF313805.

Family and domain databases

InterProiIPR010376. DUF971.
IPR003819. TauD/TfdA-like.
IPR012776. Trimethyllysine_dOase.
[Graphical view]
PfamiPF06155. DUF971. 1 hit.
PF02668. TauD. 1 hit.
[Graphical view]
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

Cross-referencesi

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.

3D structure databases

ProteinModelPortaliQ91ZW6.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiQ91ZW6. 1 interactor.
STRINGi10116.ENSRNOP00000044070.

PTM databases

iPTMnetiQ91ZW6.
PhosphoSitePlusiQ91ZW6.

Proteomic databases

PaxDbiQ91ZW6.
PRIDEiQ91ZW6.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

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]

Organism-specific databases

CTDi55217.
RGDi620629. Tmlhe.

Phylogenomic databases

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

Enzyme and pathway databases

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

Miscellaneous databases

PROiQ91ZW6.

Gene expression databases

BgeeiENSRNOG00000000729.
GenevisibleiQ91ZW6. RN.

Family and domain databases

InterProiIPR010376. DUF971.
IPR003819. TauD/TfdA-like.
IPR012776. Trimethyllysine_dOase.
[Graphical view]
PfamiPF06155. DUF971. 1 hit.
PF02668. TauD. 1 hit.
[Graphical view]
TIGRFAMsiTIGR02410. carnitine_TMLD. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiTMLH_RAT
AccessioniPrimary (citable) accession number: Q91ZW6
Secondary accession number(s): Q5U2P7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: June 20, 2002
Last sequence update: November 28, 2006
Last modified: November 2, 2016
This is version 111 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  2. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.