| Protein Name | gamma-butyrobetaine hydroxylase 1 |
|---|---|
| Organism | Homo sapiens |
| Gene ID | 8424 |
| Gene Symbol | BBOX1 |
| UniProt | O75936 (BODG_HUMAN) |
| Relationships |
Total Number of functionally related compound(s) :
73
Total Number of Articles : 49 |
Description |
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] |
| synonyms |
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| Properties | |