NameTrimethyllysine dioxygenase, mitochondrial
Synonyms
  • 1.14.11.8
  • Epsilon-trimethyllysine 2-oxoglutarate dioxygenase
  • Epsilon-trimethyllysine hydroxylase
  • TML dioxygenase
  • TML hydroxylase
  • TML-alpha-ketoglutarate dioxygenase
  • TMLD
  • TMLH
Gene NameTMLHE
OrganismHuman
Amino acid sequence
>lcl|BSEQ0004359|Trimethyllysine dioxygenase, mitochondrial
MWYHRLSHLHSRLQDLLKGGVIYPALPQPNFKSLLPLAVHWHHTASKSLTCAWQQHEDHF
ELKYANTVMRFDYVWLRDHCRSASCYNSKTHQRSLDTASVDLCIKPKTIRLDETTLFFTW
PDGHVTKYDLNWLVKNSYEGQKQKVIQPRILWNAEIYQQAQVPSVDCQSFLETNEGLKKF
LQNFLLYGIAFVENVPPTQEHTEKLAERISLIRETIYGRMWYFTSDFSRGDTAYTKLALD
RHTDTTYFQEPCGIQVFHCLKHEGTGGRTLLVDGFYAAEQVLQKAPEEFELLSKVPLKHE
YIEDVGECHNHMIGIGPVLNIYPWNKELYLIRYNNYDRAVINTVPYDVVHRWYTAHRTLT
IELRRPENEFWVKLKPGRVLFIDNWRVLHGRECFTGYRQLCGCYLTRDDVLNTARLLGLQ
A
Number of residues421
Molecular Weight49517.2
Theoretical pI7.79
GO Classification
Functions
  • trimethyllysine dioxygenase activity
  • iron ion binding
  • oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen
  • L-ascorbic acid binding
Processes
  • negative regulation of oxidoreductase activity
  • cellular nitrogen compound metabolic process
  • small molecule metabolic process
  • oxidation-reduction process
  • branched-chain amino acid catabolic process
  • carnitine biosynthetic process
Components
  • mitochondrion
  • mitochondrial matrix
General FunctionTrimethyllysine dioxygenase activity
Specific FunctionConverts trimethyllysine (TML) into hydroxytrimethyllysine (HTML).
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID15553435
UniProtKB IDQ9NVH6
UniProtKB Entry NameTMLH_HUMAN
Cellular LocationMitochondrion matrix
Gene sequence
>lcl|BSEQ0011603|Trimethyllysine dioxygenase, mitochondrial (TMLHE)
ATGTGGTACCACAGATTGTCCCACCTACACAGCAGGCTTCAGGACTTGCTGAAGGGAGGA
GTCATATATCCGGCCCTTCCACAGCCCAACTTCAAAAGCTTACTTCCTTTAGCTGTCCAT
TGGCACCATACAGCCTCCAAGTCTCTGACTTGTGCTTGGCAGCAACATGAAGATCATTTT
GAGCTGAAATATGCTAATACCGTGATGCGCTTTGATTACGTCTGGCTTCGAGACCACTGC
CGCTCAGCATCGTGCTACAACTCTAAGACTCACCAGCGCAGCCTGGATACTGCCAGTGTG
GATTTATGTATCAAGCCAAAGACCATTCGTCTGGATGAGACCACACTCTTTTTCACTTGG
CCAGATGGTCATGTGACTAAATATGATTTGAATTGGCTGGTGAAAAACAGCTATGAAGGG
CAGAAACAAAAAGTCATCCAGCCTAGAATACTATGGAATGCTGAAATCTACCAGCAAGCC
CAAGTTCCATCGGTAGATTGCCAGAGCTTCTTAGAAACCAACGAGGGACTGAAGAAGTTT
CTGCAAAACTTTCTGCTCTATGGAATTGCATTCGTAGAAAATGTCCCTCCCACTCAAGAG
CACACAGAGAAGTTGGCAGAAAGGATCAGCTTAATCAGAGAAACCATTTATGGGAGGATG
TGGTATTTCACTTCAGACTTCTCCAGAGGTGACACTGCGTACACCAAGCTAGCTCTGGAT
CGGCACACTGACACTACCTATTTTCAAGAGCCCTGTGGCATTCAAGTGTTTCATTGTCTT
AAACATGAAGGAACTGGTGGCAGGACACTGCTAGTAGATGGATTCTATGCAGCAGAACAG
GTACTTCAAAAGGCACCTGAGGAATTTGAACTCCTCAGTAAAGTGCCATTGAAGCATGAA
TATATTGAAGATGTTGGAGAATGTCACAACCACATGATTGGGATTGGGCCAGTCTTAAAT
ATCTACCCATGGAATAAAGAGCTGTATTTGATCAGATTATTCAAAGAAAAACAAAACACG
GTCAACAGGCAGTGGAACTCCTCACTCCAATGTGATATTCCTGAGAGAATATTGACTTAT
CGTCACTTCGTCTCTGGGACAAGTATTGAACATAGGGGAAGCCTTATATAA
GenBank Gene IDAF373407
GeneCard IDNot Available
GenAtlas IDTMLHE
HGNC IDHGNC:18308
Chromosome LocationX
LocusXq28
References
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