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Identification
HMDB Protein ID HMDBP03654
Secondary Accession Numbers
  • 9242
Name Trimethyllysine dioxygenase, mitochondrial
Synonyms
  1. Epsilon-trimethyllysine 2-oxoglutarate dioxygenase
  2. Epsilon-trimethyllysine hydroxylase
  3. TML dioxygenase
  4. TML hydroxylase
  5. TML-alpha-ketoglutarate dioxygenase
  6. TMLD
Gene Name TMLHE
Protein Type Unknown
Biological Properties
General Function Involved in oxidation reduction
Specific Function Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML).
Pathways
  • carnitine biosynthesis
  • Carnitine Synthesis
  • Lysine degradation
Reactions
N6,N6,N6-Trimethyl-L-lysine + Oxoglutaric acid + Oxygen → 3-hydroxy-N(6),N(6),N(6)-trimethyl-L-lysine + Succinic acid + CO(2) details
N6,N6,N6-Trimethyl-L-lysine + Oxoglutaric acid + Oxygen → 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine + Succinic acid + Carbon dioxide details
GO Classification
Biological Process
negative regulation of oxidoreductase activity
carnitine biosynthetic process
Cellular Component
mitochondrial matrix
mitochondrion
Function
ion binding
cation binding
metal ion binding
binding
catalytic activity
transition metal ion binding
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, 2-oxoglutarate as one donor, and incorporation of one atom each of oxygen into both donors
l-ascorbic acid binding
iron ion binding
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen
vitamin binding
oxidoreductase activity
trimethyllysine dioxygenase activity
Molecular Function
trimethyllysine dioxygenase activity
oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen
L-ascorbic acid binding
iron ion binding
Process
metabolic process
cellular metabolic process
betaine metabolic process
carnitine metabolic process
carnitine biosynthetic process
oxidation reduction
cellular amino acid derivative metabolic process
cellular biogenic amine metabolic process
cellular amino acid and derivative metabolic process
Cellular Location
  1. Mitochondrion matrix
Gene Properties
Chromosome Location X
Locus Xq28
SNPs TMLHE
Gene Sequence
>1266 bp
ATGTGGTACCACAGATTGTCCCACCTACACAGCAGGCTTCAGGACTTGCTGAAGGGAGGA
GTCATATATCCGGCCCTTCCACAGCCCAACTTCAAAAGCTTACTTCCTTTAGCTGTCCAT
TGGCACCATACAGCCTCCAAGTCTCTGACTTGTGCTTGGCAGCAACATGAAGATCATTTT
GAGCTGAAATATGCTAATACCGTGATGCGCTTTGATTACGTCTGGCTTCGAGACCACTGC
CGCTCAGCATCGTGCTACAACTCTAAGACTCACCAGCGCAGCCTGGATACTGCCAGTGTG
GATTTATGTATCAAGCCAAAGACCATTCGTCTGGATGAGACCACACTCTTTTTCACTTGG
CCAGATGGTCATGTGACTAAATATGATTTGAATTGGCTGGTGAAAAACAGCTATGAAGGG
CAGAAACAAAAAGTCATCCAGCCTAGAATACTATGGAATGCTGAAATCTACCAGCAAGCC
CAAGTTCCATCGGTAGATTGCCAGAGCTTCTTAGAAACCAACGAGGGACTGAAGAAGTTT
CTGCAAAACTTTCTGCTCTATGGAATTGCATTCGTAGAAAATGTCCCTCCCACTCAAGAG
CACACAGAGAAGTTGGCAGAAAGGATCAGCTTAATCAGAGAAACCATTTATGGGAGGATG
TGGTATTTCACTTCAGACTTCTCCAGAGGTGACACTGCGTACACCAAGCTAGCTCTGGAT
CGGCACACTGACACTACCTATTTTCAAGAGCCCTGTGGCATTCAAGTGTTTCATTGTCTT
AAACATGAAGGAACTGGTGGCAGGACACTGCTAGTAGATGGATTCTATGCAGCAGAACAG
GTACTTCAAAAGGCACCTGAGGAATTTGAACTCCTCAGTAAAGTGCCATTGAAGCATGAA
TATATTGAAGATGTTGGAGAATGTCACAACCACATGATTGGGATTGGGCCAGTCTTAAAT
ATCTACCCATGGAATAAAGAGCTGTATTTGATCAGGTACAACAACTATGACCGGGCTGTC
ATCAATACCGTTCCTTATGATGTCGTCCATCGCTGGTATACAGCACACCGGACTCTAACG
ATAGAGTTGAGGAGACCTGAGAATGAGTTTTGGGTCAAACTAAAGCCTGGCAGGGTCCTA
TTTATAGACAACTGGCGTGTCCTACATGGCAGGGAATGCTTCACTGGCTACCGCCAACTG
TGTGGCTGCTATTTAACAAGAGATGATGTATTAAACACTGCTCGCCTCTTGGGGCTTCAG
GCTTAA
Protein Properties
Number of Residues 421
Molecular Weight 44048.895
Theoretical pI 7.448
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Trimethyllysine dioxygenase, mitochondrial
MWYHRLSHLHSRLQDLLKGGVIYPALPQPNFKSLLPLAVHWHHTASKSLTCAWQQHEDHF
ELKYANTVMRFDYVWLRDHCRSASCYNSKTHQRSLDTASVDLCIKPKTIRLDETTLFFTW
PDGHVTKYDLNWLVKNSYEGQKQKVIQPRILWNAEIYQQAQVPSVDCQSFLETNEGLKKF
LQNFLLYGIAFVENVPPTQEHTEKLAERISLIRETIYGRMWYFTSDFSRGDTAYTKLALD
RHTDTTYFQEPCGIQVFHCLKHEGTGGRTLLVDGFYAAEQVLQKAPEEFELLSKVPLKHE
YIEDVGECHNHMIGIGPVLNIYPWNKELYLIRYNNYDRAVINTVPYDVVHRWYTAHRTLT
IELRRPENEFWVKLKPGRVLFIDNWRVLHGRECFTGYRQLCGCYLTRDDVLNTARLLGLQ
A
GenBank ID Protein Not Available
UniProtKB/Swiss-Prot ID Q9NVH6
UniProtKB/Swiss-Prot Entry Name TMLH_HUMAN
PDB IDs Not Available
GenBank Gene ID AF373407
GeneCard ID TMLHE
GenAtlas ID TMLHE
HGNC ID HGNC:18308
References
General References
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