gamma-butyrobetaine hydroxylase (gamma-butyrobetaine-2-oxoglutarate dioxygenase, gamma-butyrobetaine hydroxylase, gamma-BBH, butyrobetaine-gamma-2-oxoglutarate dioxygenase 1, BBOX1)

Function

• catalyzes the formation of L-carnitine from gamma-butyrobetaine
• amine & polyamine biosynthesis; carnitine biosynthesis

4-trimethylammoniobutanoate + 2-oxoglutarate + O2
                          <-->
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2

Cofactor: iron, ascorbate

Structure

belongs to the gamma-BBH/TMLD family

Compartment

cytoplasm

Expression

• highly expressed in kidney
• moderately expressed in liver
• very low expression in brain

More general terms

• hydroxylase; monooxygenase
• dioxygenase
• metalloprotein

Additional terms

• carnitine biosynthesis

Database

• OMIM: https://mirror.omim.org/entry/603312
• UniProt: http://www.uniprot.org/uniprot/O75936.html