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


BBOX1 (may also be known as: None)




This gene encodes gamma butyrobetaine hydroxylase which catalyzes the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthetic pathway. Carnitine is essential for the transport of activated fatty acids across the mitochondrial membrane during mitochondrial beta-oxidation. [provided by RefSeq, Jul 2008]


gbh-1 Caenorhabditis elegans
CG14630 Drosophila melanogaster
bbox1 Danio rerio
Bbox1 Mus musculus
Bbox1 Rattus norvegicus
AIM17 Saccharomyces cerevisiae

Links to external resources

Changes associated with this gene

GO Terms

GO IDGO TermGO Category
GO:0034641 cellular nitrogen compound metabolic process biological_process
GO:0044281 small molecule metabolic process biological_process
GO:0045329 carnitine biosynthetic process biological_process
GO:0005737 cytoplasm cellular_component
GO:0005739 mitochondrion cellular_component
GO:0005829 cytosol cellular_component
GO:0015629 actin cytoskeleton cellular_component
GO:0043231 intracellular membrane-bounded organelle cellular_component
GO:0005506 iron ion binding molecular_function
GO:0008270 zinc ion binding molecular_function
GO:0008336 gamma-butyrobetaine dioxygenase activity molecular_function
GO:0016491 oxidoreductase activity molecular_function
GO:0016702 oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen molecular_function
GO:0046872 metal ion binding molecular_function