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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.
Protein class

Enzymes, Metabolic proteins

Predicted location

Intracellular

Single cell type specificity

Cell type enriched (Proximal tubular cells)

Immune cell specificity

Not detected in immune cells

Cell line specificity

Group enriched (hTCEpi, RPTEC TERT1)

Molecular function

Dioxygenase, Oxidoreductase

More Types Infomation

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For Research Use Only. Not For Clinical Use.

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