trimethyllysine hydroxylase (TMLHE, trimethyllysine dioxygenase mitochondrial, epsilon-trimethyllysine 2-oxoglutarate dioxygenase, TML-alpha-ketoglutarate dioxygenase, TML hydroxylase, TML dioxygenase, TMLD)

Function

• converts trimethyllysine into hydroxytrimethyllysine
• amine & polyamine biosynthesis, carnitine biosynthesis

N(6),N(6),N(6)-trimethyl-L-lysine + 2-oxoglutarate + O2
                   <-->
3-hydroxy-N(6),N(6),N(6)-trimethyl-L-lysine + succinate + CO2

Cofactor: iron, ascorbate

Structure

• homodimer (putative)
• belongs to the gamma-BBH/TMLD family
• binding sites for ascorbate & Fe+2 are presumed from analogy with gamma-butyrobetaine hydroxylase

Compartment

mitochondrial matrix (putative)

Alternative splicing

named isoforms=2

More general terms

• hydroxylase; monooxygenase
• dioxygenase
• metalloprotein
• mitochondrial protein

Additional terms

• carnitine biosynthesis

Database

• UniProt: http://www.uniprot.org/uniprot/Q9NVH6.html