BBOX1 Catalyzes the formation of L-carnitine from gamma-butyrobetaine. Belongs to the gamma-BBH/TMLD family. Highly expressed in kidney; moderately expressed in liver; very low expression in brain. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - lysine degradation; EC; Oxidoreductase
Chromosomal Location of human Ortholog: 11p14.2
Cellular Component:  cytosol; mitochondrion
Molecular Function:  gamma-butyrobetaine dioxygenase activity; identical protein binding; iron ion binding; protein binding; zinc ion binding
Biological Process:  carnitine biosynthetic process
Reference #:  O75936 (UniProtKB)
Alt. Names/Synonyms: BBH; BBOX; BBOX1; BODG; butyrobetaine (gamma), 2-oxoglutarate dioxygenase (gamma-butyrobetaine hydroxylase) 1; G-BBH; Gamma-BBH; Gamma-butyrobetaine dioxygenase; Gamma-butyrobetaine hydroxylase; gamma-butyrobetaine hydroxylase 1; Gamma-butyrobetaine,2-oxoglutarate dioxygenase; gamma-butyrobetaine,2-oxoglutarate dioxygenase 1
Gene Symbols: BBOX1
Molecular weight: 44,715 Da
Basal Isoelectric point: 6.28  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

Cross-references to other databases:  AlphaFold  |  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  RCSB PDB  |  ENZYME  |  Phospho3D  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene