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Protein

Trimethyllysine dioxygenase, mitochondrial

Gene

Tmlhe

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

Functioni

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

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

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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi

Organism-specific databases

MGIiMGI:2180203 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_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
MINTiQ91ZE0
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
OMAiKRRMCGA
OrthoDBiEOG091G062L
PhylomeDBiQ91ZE0
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

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

Entry informationi

Entry nameiTMLH_MOUSE
AccessioniPrimary (citable) accession number: Q91ZE0
Secondary accession number(s): Q3TSX6, Q3UMX1, Q91XH1
Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 20, 2002
Last sequence update: June 20, 2002
Last modified: March 28, 2018
This is version 131 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, 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

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