CAG33093.1
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
BBOX1
NCBI Official Synonym Full Names
butyrobetaine (gamma), 2-oxoglutarate dioxygenase (gamma-butyrobetaine hydroxylase) 1
NCBI Protein Information
gamma-butyrobetaine dioxygenase
UniProt Protein Name
Gamma-butyrobetaine dioxygenase
UniProt Synonym Protein Names
Gamma-butyrobetaine hydroxylase; Gamma-BBH; Gamma-butyrobetaine,2-oxoglutarate dioxygenase
UniProt Synonym Gene Names
UniProt Entry Name
BODG_HUMAN
NCBI Summary for BBOX1
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]
UniProt Comments for BBOX1
BBOX1: Catalyzes the formation of L-carnitine from gamma- butyrobetaine. Belongs to the gamma-BBH/TMLD family.
Protein type: EC 1.14.11.1; Amino Acid Metabolism - lysine degradation; Oxidoreductase
Chromosomal Location of Human Ortholog: 11p14.2
Cellular Component: mitochondrion; cytosol
Molecular Function: gamma-butyrobetaine dioxygenase activity; zinc ion binding; iron ion binding
Biological Process: carnitine biosynthetic process
Research Articles on BBOX1
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Products associated with anti-BBOX1 antibody
Pathways associated with anti-BBOX1 antibody
Diseases associated with anti-BBOX1 antibody
Organs/Tissues associated with anti-BBOX1 antibody
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