Gamma-butyrobetaine dioxygenase

A gamma-butyrobetaine dioxygenase that is encoded in the genome of human. [PRO:DNx, UniProtKB:O75936]

EC 1.14.11.1;
Gamma-butyrobetaine hydroxylase;
Gamma-BBH;
Gamma-butyrobetaine,2-oxoglutarate dioxygenase

Research

Drug	Taxonomy	Measurement	Average (mM)	Bioassay(s)	Publication(s)
ebselen	Homo sapiens (human)	IC50	0.7420	5	5
succinylacetone	Homo sapiens (human)	IC50	100.0000	1	1
thiram	Homo sapiens (human)	IC50	23.5400	5	5
daminozide	Homo sapiens (human)	IC50	100.0000	1	1
diphenyldiselenide	Homo sapiens (human)	IC50	46.4000	5	5
benzeneseleninic acid	Homo sapiens (human)	IC50	11.9200	5	5
3-(2,2,2-trimethylhydrazine)propionate	Homo sapiens (human)	IC50	62.0000	1	1
3-(2,2,2-trimethylhydrazine)propionate	Homo sapiens (human)	Ki	16.0000	1	1
crotonic acid betaine	Homo sapiens (human)	IC50	3.8000	1	1

Drug	Taxonomy	Measurement	Average (mM)	Bioassay(s)	Publication(s)
ebselen	Homo sapiens (human)	Kd	4.1000	2	2
thiram	Homo sapiens (human)	Kd	47.0000	2	2
diphenyldiselenide	Homo sapiens (human)	Kd	200.0000	2	2
benzeneseleninic acid	Homo sapiens (human)	Kd	9.8000	2	2

This protein enables 5 target(s):

Target	Category	Definition
iron ion binding	molecular function	Binding to an iron (Fe) ion. [GOC:ai]
protein binding	molecular function	Binding to a protein. [GOC:go_curators]
zinc ion binding	molecular function	Binding to a zinc ion (Zn). [GOC:ai]
gamma-butyrobetaine dioxygenase activity	molecular function	Catalysis of the reaction: 2-oxoglutarate + 4-(trimethylammonio)butanoate + O2 = carnitine + CO2 + succinate. [EC:1.14.11.1, RHEA:24028]
identical protein binding	molecular function	Binding to an identical protein or proteins. [GOC:jl]

This protein is located in 2 target(s):

Target	Category	Definition
cytosol	cellular component	The part of the cytoplasm that does not contain organelles but which does contain other particulate matter, such as protein complexes. [GOC:hjd, GOC:jl]
extracellular exosome	cellular component	A vesicle that is released into the extracellular region by fusion of the limiting endosomal membrane of a multivesicular body with the plasma membrane. Extracellular exosomes, also simply called exosomes, have a diameter of about 40-100 nm. [GOC:BHF, GOC:mah, GOC:vesicles, PMID:15908444, PMID:17641064, PMID:19442504, PMID:19498381, PMID:22418571, PMID:24009894]

This protein is active in 1 target(s):

Target	Category	Definition
mitochondrion	cellular component	A semiautonomous, self replicating organelle that occurs in varying numbers, shapes, and sizes in the cytoplasm of virtually all eukaryotic cells. It is notably the site of tissue respiration. [GOC:giardia, ISBN:0198506732]

This protein is involved in 1 target(s):

Target	Category	Definition
carnitine biosynthetic process	biological process	The chemical reactions and pathways resulting in the formation of carnitine (hydroxy-trimethyl aminobutyric acid), a compound that participates in the transfer of acyl groups across the inner mitochondrial membrane. [GOC:jl, ISBN:0198506732]