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Protein

Trimethyllysine dioxygenase, mitochondrial

Gene

Tmlhe

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML).By similarity

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

  • carnitine biosynthetic process Source: MGI
Complete GO annotation...

Keywords - Molecular functioni

Dioxygenase, Oxidoreductase

Keywords - Biological processi

Carnitine biosynthesis

Keywords - Ligandi

Iron, Metal-binding

Enzyme and pathway databases

BRENDAi1.14.11.8. 3474.
ReactomeiR-MMU-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
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Unplaced

Organism-specific databases

MGIiMGI:2180203. Tmlhe.

Subcellular locationi

  • Mitochondrion matrix By similarity

GO - Cellular componenti

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

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 15MitochondrionBy similarityAdd BLAST15
ChainiPRO_000000279616 – 421Trimethyllysine dioxygenase, mitochondrialAdd BLAST406

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei179N6-acetyllysineCombined sources1
Modified residuei236N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ91ZE0.
MaxQBiQ91ZE0.
PaxDbiQ91ZE0.
PeptideAtlasiQ91ZE0.
PRIDEiQ91ZE0.

PTM databases

iPTMnetiQ91ZE0.
PhosphoSitePlusiQ91ZE0.
SwissPalmiQ91ZE0.

Expressioni

Developmental stagei

Present already at 9.0 dpc. At 10.5 dpc, expressed throughout the embryo. At 12.5 dpc, higher levels in the developing lung, liver and brain compared to other tissues. In the postnatal day 7 brain, high levels in the Purkinje cell layer of the cerebellum and in the hyppocampal areas of the dentate gyrus and CA1, CA2 and CA3 pyramidal cells.1 Publication

Gene expression databases

BgeeiENSMUSG00000079834.
CleanExiMM_TMLHE.
GenevisibleiQ91ZE0. MM.

Interactioni

Subunit structurei

Homodimer.By similarity

Protein-protein interaction databases

IntActiQ91ZE0. 1 interactor.
MINTiMINT-4138064.
STRINGi10090.ENSMUSP00000111624.

Structurei

3D structure databases

ProteinModelPortaliQ91ZE0.
SMRiQ91ZE0.
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.
InParanoidiQ91ZE0.
KOiK00474.
OMAiIEINEFW.
OrthoDBiEOG091G062L.
PhylomeDBiQ91ZE0.
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.

Sequencei

Sequence statusi: Complete.

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

Q91ZE0-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MWYHKLLHQQ SRLRNLMKRG NIAQGLHLSN FKSLFSSSIH WCHTTSKSVN
60 70 80 90 100
CTWHQHEDHL ELQYAGTVMR FDYVWLRDHC RSASCYNSKT HQRSLDTASV
110 120 130 140 150
DLCIKPKTVH LDETMLFFTW PDGHVTRYDL DWLVKNSYEG QKQKVIQPRI
160 170 180 190 200
LWNSKLYQQA QVPSVDFQCF LETNEGLKKF LQNFLLYGIA FVENVPPTEE
210 220 230 240 250
HTEKLAERIS LIRETIYGRM WYFTSDFSRG DTAYTKLALD RHTDTTYFQE
260 270 280 290 300
PCGIQVFHCL KHEGTGGRTL LVDGFYAAQQ VLQKAPEEFE LLSKVPLKHE
310 320 330 340 350
YIENVGQCHN HMIGVGPILN IYPWNKELYL IRYNNYDRAV INTVPYDVVH
360 370 380 390 400
RWYTAHRTLT TELRRPENEL WVKLKPGKVL FIDNWRVLHG RESFTGYRQL
410 420
CGCYLTRDDV LNTARLLGLH A
Length:421
Mass (Da):49,610
Last modified:June 20, 2002 - v2
Checksum:i1474AD5742E88F43
GO

Sequence cautioni

The sequence AAK54387 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti8H → Q in BAE36549 (PubMed:16141072).Curated1
Sequence conflicti188G → K in BAE36549 (PubMed:16141072).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK144627 mRNA. Translation: BAE25977.1.
AK161724 mRNA. Translation: BAE36549.1.
CAAA01137825 Genomic DNA. No translation available.
CAAA01183048 Genomic DNA. No translation available.
CAAA01120074 Genomic DNA. No translation available.
CAAA01019119 Genomic DNA. No translation available.
CH466822 Genomic DNA. Translation: EDL07804.1.
BC010495 mRNA. Translation: AAH10495.1.
BC115365 mRNA. Translation: AAI15366.1.
AY033513 mRNA. Translation: AAK54387.1. Different initiation.
RefSeqiNP_620097.1. NM_138758.1.
UniGeneiMm.394228.

Genome annotation databases

EnsembliENSMUST00000115964; ENSMUSP00000111624; ENSMUSG00000079834.
GeneIDi192289.
KEGGimmu:192289.
UCSCiuc009uyo.1. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK144627 mRNA. Translation: BAE25977.1.
AK161724 mRNA. Translation: BAE36549.1.
CAAA01137825 Genomic DNA. No translation available.
CAAA01183048 Genomic DNA. No translation available.
CAAA01120074 Genomic DNA. No translation available.
CAAA01019119 Genomic DNA. No translation available.
CH466822 Genomic DNA. Translation: EDL07804.1.
BC010495 mRNA. Translation: AAH10495.1.
BC115365 mRNA. Translation: AAI15366.1.
AY033513 mRNA. Translation: AAK54387.1. Different initiation.
RefSeqiNP_620097.1. NM_138758.1.
UniGeneiMm.394228.

3D structure databases

ProteinModelPortaliQ91ZE0.
SMRiQ91ZE0.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiQ91ZE0. 1 interactor.
MINTiMINT-4138064.
STRINGi10090.ENSMUSP00000111624.

PTM databases

iPTMnetiQ91ZE0.
PhosphoSitePlusiQ91ZE0.
SwissPalmiQ91ZE0.

Proteomic databases

EPDiQ91ZE0.
MaxQBiQ91ZE0.
PaxDbiQ91ZE0.
PeptideAtlasiQ91ZE0.
PRIDEiQ91ZE0.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000115964; ENSMUSP00000111624; ENSMUSG00000079834.
GeneIDi192289.
KEGGimmu:192289.
UCSCiuc009uyo.1. mouse.

Organism-specific databases

CTDi55217.
MGIiMGI:2180203. Tmlhe.

Phylogenomic databases

eggNOGiKOG3889. Eukaryota.
COG2175. LUCA.
GeneTreeiENSGT00530000063582.
HOGENOMiHOG000210004.
HOVERGENiHBG035650.
InParanoidiQ91ZE0.
KOiK00474.
OMAiIEINEFW.
OrthoDBiEOG091G062L.
PhylomeDBiQ91ZE0.
TreeFamiTF313805.

Enzyme and pathway databases

UniPathwayiUPA00118.
BRENDAi1.14.11.8. 3474.
ReactomeiR-MMU-71262. Carnitine synthesis.

Miscellaneous databases

PROiQ91ZE0.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000079834.
CleanExiMM_TMLHE.
GenevisibleiQ91ZE0. MM.

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_MOUSE
AccessioniPrimary (citable) accession number: Q91ZE0
Secondary accession number(s): Q3TSX6, Q3UMX1, Q91XH1
Entry historyi
Integrated into UniProtKB/Swiss-Prot: June 20, 2002
Last sequence update: June 20, 2002
Last modified: November 2, 2016
This is version 124 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, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. 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.