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BBOX1

Function

Catalyzes the formation of L-carnitine from gamma-butyrobetaine.

Pathway

Amine and polyamine biosynthesis; carnitine biosynthesis.

Sequence Similarities

Belongs to the gamma-BBH/TMLD family.

Tissue Specificity

Highly expressed in kidney; moderately expressed in liver; very low expression in brain.

Cellular localization

Alternative names

BBH, BBOX, BBOX1, Gamma-butyrobetaine dioxygenase, Gamma-butyrobetaine hydroxylase, Gamma-BBH

swissprot:O75936 omim:603312 entrezGene:8424