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Q91ZW6 (TMLH_RAT) Reviewed, UniProtKB/Swiss-Prot

Last modified November 16, 2011. Version 76. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (2) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order
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Names and origin

Protein namesRecommended 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 names
Name:Tmlhe
Synonyms:Tmlh
OrganismRattus norvegicus (Rat)
Taxonomic identifier10116 [NCBI]
Taxonomic lineageEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Sciurognathi › Muroidea › Muridae › Murinae › Rattus

Protein attributes

Sequence length421 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML).

Catalytic activity

N6,N6,N(6)-trimethyl-L-lysine + 2-oxoglutarate + O2 = 3-hydroxy-N6,N6,N(6)-trimethyl-L-lysine + succinate + CO2.

Cofactor

Binds 1 Fe2+ ion per subunit By similarity.

Ascorbate.

Pathway

Amine and polyamine biosynthesis; carnitine biosynthesis.

Subunit structure

Homodimer.

Subcellular location

Mitochondrion matrix.

Sequence similarities

Belongs to the gamma-BBH/TMLD family.

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]
Isoform 1 (identifier: Q91ZW6-1)

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.
Isoform 2 (identifier: Q91ZW6-2)

The sequence of this isoform differs from the canonical sequence as follows:
     62-62: L → LGTKPRALRLLEL
Note: No experimental confirmation available.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Transit peptide1 – 1515Mitochondrion By similarity
Chain16 – 421406Trimethyllysine dioxygenase, mitochondrial
PRO_0000002797

Sites

Metal binding2421Iron; catalytic By similarity
Metal binding2441Iron; catalytic By similarity
Metal binding3891Iron; catalytic By similarity

Amino acid modifications

Modified residue2361N6-acetyllysine By similarity

Natural variations

Alternative sequence621L → LGTKPRALRLLEL in isoform 2.
VSP_021580

Sequences

Sequence LengthMass (Da)Tools
Isoform 1 [UniParc].

Last modified November 28, 2006. Version 2.
Checksum: 487AB5FCA0D02D25

FASTA42149,568
        10         20         30         40         50         60 
MWYHKLLHQQ SRLQNLMKRG DIAHGLRLSG FKSLFPFSLH WCHTASKSVN CTWHQHEDHL 

        70         80         90        100        110        120 
ELQYASTVMR FDYVWLRDHC RSASCYNSKT HQRSLDTASV DLCIKPKTIR LDESTLFFTW 

       130        140        150        160        170        180 
PDGHVTRYDL DWLVKNSYEG QKQEVIQPRV LWNAKLYQDA QLPSVDFQGF LETKEGLKKF 

       190        200        210        220        230        240 
LQNFLLYGIA FVENVPPTQE HTEKLARRVS LIRETIYGRM WYFTSDFSRG DTAYTKLALD 

       250        260        270        280        290        300 
RHTDTTYFQE PCGIQVFHCL KHEGTGGRTL LVDGFYAAQQ VLQRAPEEFD LLSQVPLKHE 

       310        320        330        340        350        360 
YIENVGQCHN HMIGVGPILN IYPWNKELYL IRYNNYDRAV INTVPYDVVR RWYAAHRTLT 

       370        380        390        400        410        420 
TELRRPENEL WVKLKPGKVL FIDNWRVLHG RESFTGYRQL CGCYLTRDDV LNTARILGLH 


A

« Hide

Isoform 2 [UniParc].

Checksum: F363DFA37363D692
Show »

FASTA43350,916

References

« Hide 'large scale' references
[1]"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res. 14:2121-2127(2004) [PubMed: 15489334] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2).
Tissue: Kidney.
[2]"Molecular and biochemical characterization of rat epsilon-N-trimethyllysine hydroxylase, the first enzyme of carnitine biosynthesis."
Vaz F.M., Ofman R., Westinga K., Back J.W., Wanders R.J.A.
J. Biol. Chem. 276:33512-33517(2001) [PubMed: 11431483] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 17-421 (ISOFORM 1), PARTIAL PROTEIN SEQUENCE, CHARACTERIZATION.
Strain: Wistar.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ		BC085923 mRNA. Translation: AAH85923.1.
AF374406 mRNA. Translation: AAL01252.1.
IPIIPI00327338.
IPI00568124.
RefSeqNP_596878.2. NM_133387.2.
UniGeneRn.3607.

3D structure databases

ProteinModelPortalQ91ZW6.
ModBaseSearch...

Protein-protein interaction databases

IntActQ91ZW6. 1 interaction.

Proteomic databases

PRIDEQ91ZW6.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENSRNOT00000000956; ENSRNOP00000000956; ENSRNOG00000000729.
ENSRNOT00000049573; ENSRNOP00000044070; ENSRNOG00000000729.
GeneID170898.
KEGGrno:170898.
NMPDRfig|10116.3.peg.17238.
UCSCAF374406. rat.

Organism-specific databases

CTD55217.
RGD620629. Tmlhe.

Phylogenomic databases

eggNOGmaNOG08663.
GeneTreeENSGT00530000063582.
HOVERGENHBG035650.
OMALCGCYLT.
PhylomeDBQ91ZW6.

Enzyme and pathway databases

BioCycMetaCyc:MONOMER-14429.

Gene expression databases

ArrayExpressQ91ZW6.
GenevestigatorQ91ZW6.
GermOnlineENSRNOG00000000729. Rattus norvegicus.

Family and domain databases

InterProIPR010376. DUF971.
IPR003819. Taurine_dOase.
IPR012776. Trimethyllysine_dOase.
[Graphical view]
KOK00474.
PfamPF06155. DUF971. 1 hit.
PF02668. TauD. 1 hit.
[Graphical view]
TIGRFAMsTIGR02410. Carnitine_TMLD. 1 hit.
ProtoNetSearch...

Other

NextBio621309.

Entry information

Entry nameTMLH_RAT
AccessionPrimary (citable) accession number: Q91ZW6
Secondary accession number(s): Q5U2P7
Entry history
Integrated into UniProtKB/Swiss-Prot: June 20, 2002
Last sequence update: November 28, 2006
Last modified: November 16, 2011
This is version 76 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Relevant documents

PATHWAY comments

Index of metabolic and biosynthesis pathways

SIMILARITY comments

Index of protein domains and families

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