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