Names & Taxonomy

Uniprot ID:
O75936
Entry Name:
BODG_HUMAN
Status:
reviewed
Protein Names:
Gamma-butyrobetaine dioxygenase (EC 1.14.11.1) (Gamma-butyrobetaine hydroxylase) (Gamma-BBH) (Gamma-butyrobetaine,2-oxoglutarate dioxygenase)
Gene Names:
BBOX1 BBH BBOX
Gene Names Primary:
BBOX1
Organism:
Homo sapiens (Human)

Structure

Length:
387
Sequence:
MACTIQKAEALDGAHLMQILWYDEEESLYPAVWLRDNCPCSDCYLDSAKARKLLVEALDVNIGIKGLIFDRKKVYITWPDEHYSEFQADWLKKRCFSKQARAKLQRELFFPECQYWGSELQLPTLDFEDVLRYDEHAYKWLSTLKKVGIVRLTGASDKPGEVSKLGKRMGFLYLTFYGHTWQVQDKIDANNVAYTTGKLSFHTDYPALHHPPGVQLLHCIKQTVTGGDSEIVDGFNVCQKLKKNNPQAFQILSSTFVDFTDIGVDYCDFSVQSKHKIIELDDKGQVVRINFNNATRDTIFDVPVERVQPFYAALKEFVDLMNSKESKFTFKMNPGDVITFDNWRLLHGRRSYEAGTEISRHLEGAYADWDVVMSRLRILRQRVENGN
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Cytoplasm.

Function

Function:
Catalyzes the formation of L-carnitine from gamma-butyrobetaine.
Pathway:
Amine and polyamine biosynthesis; carnitine biosynthesis.
Catalytic Activity:
4-trimethylammoniobutanoate + 2-oxoglutarate + O(2) = 3-hydroxy-4-trimethylammoniobutanoate + succinate + CO(2).
Cofactor:
COFACTOR: Name=Fe(2+); Xref=ChEBI:CHEBI:29033;
Cross Reference Drug Bank:
DB00139 DB00126
Gene Ontology Go:
cytosol
extracellular exosome
mitochondrion
gamma-butyrobetaine dioxygenase activity
iron ion binding
zinc ion binding
branched-chain amino acid catabolic process
carnitine biosynthetic process
cellular nitrogen compound metabolic process
small molecule metabolic process
Gene Ontology Biological Process:
branched-chain amino acid catabolic process
carnitine biosynthetic process
cellular nitrogen compound metabolic process
small molecule metabolic process
Gene Ontology Molecular Function:
gamma-butyrobetaine dioxygenase activity
iron ion binding
zinc ion binding
Gene Ontology Cellular Component:
cytosol
extracellular exosome
mitochondrion
Keywords:
3D-structure
Carnitine biosynthesis
Complete proteome
Cytoplasm
Dioxygenase
Iron
Metal-binding
Oxidoreductase
Phosphoprotein
Reference proteome
Zinc
Interacts With:
A0MZ66-7

Publication

PubMed ID:
9753662 14702039 15489334 20599753