FB2026_01 , released March 12, 2026
FB2026_01 , released March 12, 2026
Gene Group: GAMMA-BUTYROBETAINE HYDROXYLASES
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General Information
Name
GAMMA-BUTYROBETAINE HYDROXYLASES
Species
D. melanogaster
Symbol
GBBH
FlyBase ID
FBgg0002012
Date last reviewed
2024-10-15
Number of members
3
Description
Description
Gamma-butyrobetaine hydroxylases are 2-oxoglutarate-dependent dioxygenases that catalyze the hydroxylation of gamma-butyrobetaine to carnitine, the last and rate-limiting step in the biosynthesis of carnitine from lysine. (Adapted from PMID:9753662 and FBrf0247638.)
Notes on Group

CG5321 and CG14630 are likely to be mitochondrial based on MitoFates and TargetP mitochondrial targeting sequence (MTS) prediction and have been detected in the mitochondrion in a high-throughput proteomics study (FBrf0224590). CG10814 has no predicted MTS or experimental data supporting mitochondrial localization. FBrf0233151 suggests that CG10814 is the functional ortholog of human BBOX1 (gamma-butyrobetaine hydroxylase 1; HGNC:964), which acts in the cytosol in the carnitine biosynthesis pathway, due to its lack of a MTS (FBrf0247638).

Source Material
The GAMMA-BUTYROBETAINE HYDROXYLASES Gene Group has been compiled using the following publication(s): Carillo et al., 2020 .
Key Gene Ontology (GO) terms
Cellular Component
Enzymatic activity
Enzyme name (EC)
gamma-butyrobetaine dioxygenase (1.14.11.1)
Related Gene Groups
Members (3)
For all members:
GO ribbon stack
 molecular functionbiological processcellular component
External Data
Equivalent Group(s)
Other resource(s)
Synonyms and Secondary IDs
Synonym(s)
GBBH
GAMMA-BUTYROBETAINE HYDROXYLASES
gamma-butyrobetaine dioxygenases
Secondary FlyBase ID(s)
References (3)