| Identification |
|---|
| HMDB Protein ID
| CDBP02359 |
| Secondary Accession Numbers
| Not Available |
| Name
| Trimethyllysine dioxygenase, mitochondrial |
| Description
| Not Available |
| Synonyms
|
- Epsilon-trimethyllysine 2-oxoglutarate dioxygenase
- Epsilon-trimethyllysine hydroxylase
- TML dioxygenase
- TML hydroxylase
- TML-alpha-ketoglutarate dioxygenase
- TMLD
|
| Gene Name
| TMLHE |
| Protein Type
| Enzyme |
| Biological Properties |
|---|
| General Function
| Involved in oxidation reduction |
| Specific Function
| Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML).
|
| GO Classification
|
| Biological Process |
| negative regulation of oxidoreductase activity |
| carnitine biosynthetic process |
| Cellular Component |
| mitochondrial matrix |
| mitochondrion |
| Function |
| iron ion binding |
| oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen |
| vitamin binding |
| oxidoreductase activity |
| trimethyllysine dioxygenase activity |
| 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 |
| 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 |
| cellular biogenic amine metabolic process |
| cellular amino acid and derivative metabolic process |
| metabolic process |
| cellular metabolic process |
| betaine metabolic process |
| carnitine metabolic process |
| carnitine biosynthetic process |
| oxidation reduction |
| cellular amino acid derivative metabolic process |
|
| Cellular Location
|
- Mitochondrion matrix
|
| Pathways
|
| Name | SMPDB/Pathwhiz | KEGG | | Carnitine Synthesis |    | Not Available | | carnitine biosynthesis | Not Available | Not Available |
|
| 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
|
| External Links |
|---|
| 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
| Not Available |