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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.
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| Protein type: Amino Acid Metabolism - lysine degradation; EC 1.14.11.1; Oxidoreductase |
| Chromosomal Location of human Ortholog: 11p14.2 |
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Cellular Component:
cytosol; mitochondrion
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Molecular Function:
gamma-butyrobetaine dioxygenase activity; identical protein binding; iron ion binding; protein binding; zinc ion binding
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Biological Process:
carnitine biosynthetic process
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Reference #:
O75936
(UniProtKB)
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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
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Gene Symbols: BBOX1
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Molecular weight:
44,715 Da
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Basal Isoelectric point:
6.28
Predict pI for various phosphorylation states
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