TMLHE

Entrez Summary: This gene encodes the protein trimethyllysine dioxygenase which is the first enzyme in the carnitine biosynthesis pathway. Carnitine play an essential role in the transport of activated fatty acids across the inner mitochondrial membrane. The encoded protein converts trimethyllysine into hydroxytrimethyllysine. A pseudogene of this gene is found on chromosome X. Alternate splicing results in multiple transcript variants.[provided by RefSeq, May 2010].
UniProt Summary: Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML) (PubMed:11431483, PubMed:23092983). {ECO:0000269|PubMed:11431483, ECO:0000269|PubMed:23092983}.

Pfam Domains GO Terms

Compound Hit Most Correlated Genes in Chemogenomics Tissues where Essential in the Avana Dataset (DepMap 20Q1)

Global Fraction of Cell Lines Where Essential: 0/683

Tissue	Fraction Of Cell Lines Where Essential
1290807.0	0/1
909776.0	0/1
bile duct	0/28
blood	0/26
bone	0/25
breast	0/30
central nervous system	0/49
cervix	0/4
colorectal	0/17
esophagus	0/11
fibroblast	0/1
gastric	0/14
kidney	0/18
liver	0/19
lung	0/72
lymphocyte	0/14
ovary	0/25
pancreas	0/22
peripheral nervous system	0/15
plasma cell	0/12
prostate	0/1
skin	0/20
soft tissue	0/7
thyroid	0/2
upper aerodigestive	0/22
urinary tract	0/28
uterus	0/5

Expression Distribution

trimethyllysine dioxygenase activity
carnitine biosynthetic process
amino-acid betaine biosynthetic process
oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen
carnitine metabolic process
amino-acid betaine metabolic process
cellular modified amino acid biosynthetic process
iron ion binding
ammonium ion metabolic process
cellular modified amino acid metabolic process
mitochondrial matrix
oxidation-reduction process
mitochondrion
organonitrogen compound biosynthetic process
cellular nitrogen compound biosynthetic process