Protein Name gamma-butyrobetaine hydroxylase 1
Organism Homo sapiens
Gene ID 8424
Gene Symbol


UniProt O75936 (BODG_HUMAN)
Relationships Total Number of functionally related compound(s) : 73
Total Number of Articles : 49

gamma-butyrobetaine hydroxylase 1

Gene Summary

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]

  • gamma-butyrobetaine dioxygenase
  • butyrobetaine (gamma), 2-oxoglutarate dioxygenase (gamma-butyrobetaine hydroxylase) 1
  • gamma-butyrobetaine,2-oxoglutarate dioxygenase 1
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