Trimethyllysine dioxygenase, mitochondrial precursor

Names and origin

Protein attributes

General annotation (Comments)

Ontologies

Alternative products

Sequence annotation (Features)

Sequences

References

Cross-references

Entry information

Relevant documents

Names and origin

Protein attributes

General annotation (Comments)

Ontologies

Alternative products

Sequence annotation (Features)

Sequences

References

Cross-references

Entry information

Relevant documents

Molecule processing

Sites

Amino acid modifications

Natural variations

Molecule processing

Sites

Amino acid modifications

Natural variations

Sequence databases

3D structure databases

Protein-protein interaction databases

Proteomic databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Gene expression databases

Family and domain databases

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Reviewed, UniProtKB/Swiss-Prot Q91ZW6 (TMLH_RAT)

Last modified February 9, 2010. Version 59. History...

Clusters with 100%, 90%, 50% identity |

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Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Alternative products · Sequence annotation (Features) · Sequences · References · Cross-references · Entry information · Relevant documents

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Protein names

Recommended name:
    Trimethyllysine dioxygenase, mitochondrial
    EC=1.14.11.8
Alternative name(s):
    Epsilon-trimethyllysine 2-oxoglutarate dioxygenase
    TML-alpha-ketoglutarate dioxygenase
      Short name=TML dioxygenase
      Short name=TMLD
    TML hydroxylase

Gene names

Name:	Tmlhe
Synonyms:	Tmlh

Organism

Rattus norvegicus (Rat)

Taxonomic identifier

10116 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Sciurognathi › Muroidea › Muridae › Murinae › Rattus

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Sequence length	421 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at protein level.

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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	Iron. Ascorbate.
Pathway	Amine and polyamine biosynthesis; carnitine biosynthesis.
Subunit structure	Homodimer.
Subcellular location	Mitochondrion matrix.
Sequence similarities	Belongs to the gamma-BBH/TMLD family.

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Keywords
Biological process	Carnitine biosynthesis
Cellular component	Mitochondrion
Coding sequence diversity	Alternative splicing
Domain	Transit peptide
Ligand	Iron
Molecular function	Dioxygenase Oxidoreductase
PTM	Acetylation
Technical term	Direct protein sequencing
Gene Ontology (GO)
Biological process	carnitine biosynthetic process Ref.2 Traceable author statement. Source: RGD oxidation reduction Inferred from electronic annotation. Source: UniProtKB-KW
Cellular component	mitochondrial matrix Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular function	L-ascorbic acid binding Inferred from electronic annotation. Source: InterPro iron ion binding Inferred from electronic annotation. Source: UniProtKB-KW oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen Inferred from electronic annotation. Source: UniProtKB-KW trimethyllysine dioxygenase activity Ref.2 Inferred from direct assay. Source: RGD
Complete GO annotation...

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Feature key

Position(s)

Length

Description

Graphical view

Feature identifier

Transit peptide

1 – 15

Mitochondrion By similarity

Chain

16 – 421

406

Trimethyllysine dioxygenase, mitochondrial

PRO_0000002797

Binding site

242

2-oxoglutarate Probable

Binding site

244

2-oxoglutarate Probable

Binding site

389

2-oxoglutarate Probable

Modified residue

236

N6-acetyllysine By similarity

Alternative sequence

L → LGTKPRALRLLEL in isoform 2.

VSP_021580

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Sequence		Length	Mass (Da)
Isoform 1 [UniParc]. Last modified November 28, 2006. Version 2. Checksum: 487AB5FCA0D02D25 Show »	FASTA	421	49,568
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. Checksum: F363DFA37363D692 Show »	FASTA	433	50,916
10 20 30 40 50 60 MWYHKLLHQQ SRLQNLMKRG DIAHGLRLSG FKSLFPFSLH WCHTASKSVN CTWHQHEDHL 70 80 90 100 110 120 ELGTKPRALR LLELQYASTV MRFDYVWLRD HCRSASCYNS KTHQRSLDTA SVDLCIKPKT 130 140 150 160 170 180 IRLDESTLFF TWPDGHVTRY DLDWLVKNSY EGQKQEVIQP RVLWNAKLYQ DAQLPSVDFQ 190 200 210 220 230 240 GFLETKEGLK KFLQNFLLYG IAFVENVPPT QEHTEKLARR VSLIRETIYG RMWYFTSDFS 250 260 270 280 290 300 RGDTAYTKLA LDRHTDTTYF QEPCGIQVFH CLKHEGTGGR TLLVDGFYAA QQVLQRAPEE 310 320 330 340 350 360 FDLLSQVPLK HEYIENVGQC HNHMIGVGPI LNIYPWNKEL YLIRYNNYDR AVINTVPYDV 370 380 390 400 410 420 VRRWYAAHRT LTTELRRPEN ELWVKLKPGK VLFIDNWRVL HGRESFTGYR QLCGCYLTRD 430 DVLNTARILG LHA « Hide

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	« Hide 'large scale' referencesShow '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.
+	Additional computationally mapped references.

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Sequence databases
EMBL GenBank DDBJ	BC085923 mRNA. Translation: AAH85923.1. AF374406 mRNA. Translation: AAL01252.1.
IPI	IPI00327338. IPI00568124.
RefSeq	NP_596878.2.
UniGene	Rn.3607
3D structure databases
SMR	Q91ZW6. Positions 161-408.
ModBase	Search...
Protein-protein interaction databases
STRING	Q91ZW6.
Proteomic databases
PRIDE	Q91ZW6.
Genome annotation databases
Ensembl	ENSRNOT00000000956; ENSRNOP00000000956; ENSRNOG00000000729; Rattus norvegicus. [Genome view]
GeneID	170898.
KEGG	rno:170898.
NMPDR	fig\|10116.3.peg.17238.
UCSC	AF374406. rat.
Organism-specific databases
CTD	170898.
RGD	620629. Tmlhe.
Phylogenomic databases
eggNOG	maNOG08663.
HOVERGEN	Q91ZW6.
OMA	ELYLIRY.
PhylomeDB	Q91ZW6.
Enzyme and pathway databases
BioCyc	MetaCyc:MONOMER-14429.
BRENDA	1.14.11.8. 248.
Gene expression databases
ArrayExpress	Q91ZW6.
Genevestigator	Q91ZW6.
GermOnline	ENSRNOG00000000729. Rattus norvegicus.
Family and domain databases
InterPro	IPR003819. Taurine_dOase. IPR012776. Trimethyllysine_dOase. [Graphical view]
PANTHER	PTHR10696:SF2. tMLys_dOase. 1 hit.
Pfam	PF02668. TauD. 1 hit. [Graphical view]
TIGRFAMs	TIGR02410. carnitine_TMLD. 1 hit.
ProtoNet	Search...
Other Resources
NextBio	621309.

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Entry name

TMLH_RAT

Accession

Primary (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:	February 9, 2010
This is version 59 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)

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PATHWAY comments

Index of metabolic and biosynthesis pathways

SIMILARITY comments

Index of protein domains and families

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.