Search on: BUTYROBETAINE HYDROXYLASE 
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Descriptor English:   gamma-Butyrobetaine Dioxygenase 
Descriptor Spanish:   gamma-Butirobetaína Dioxigenasa 
Descriptor Portuguese:   gama-Butirobetaína Dioxigenase 
Synonyms English:   4-Trimethylaminobutyrate, 2-Oxoglutarate Oxygen Oxidoreductase (3-Hydroxylating)
Butyrobetaine Hydroxylase
Trimethylaminobutyrate Hydroxylase
gamma-Butyrobetaine Hydroxylase
gamma-Butyrobetaine, 2-Oxoglutarate Dioxygenase
gamma-Butyrobetaine,2-Oxoglutarate Dioxygenase
2-Oxoglutarate Dioxygenase gamma-Butyrobetaine
Dioxygenase gamma-Butyrobetaine, 2-Oxoglutarate
Dioxygenase, gamma-Butyrobetaine
Dioxygenase, gamma-Butyrobetaine,2-Oxoglutarate
Hydroxylase, Butyrobetaine
Hydroxylase, Trimethylaminobutyrate
Hydroxylase, gamma-Butyrobetaine
gamma Butyrobetaine Dioxygenase
gamma Butyrobetaine Hydroxylase
gamma Butyrobetaine, 2 Oxoglutarate Dioxygenase
gamma Butyrobetaine,2 Oxoglutarate Dioxygenase  
Tree Number:   D08.811.682.690.708.401
Definition English:   An enzyme that catalyzes the HYDROXYLATION of gamma-butyrobetaine to L-CARNITINE. It is the last enzyme in the biosynthetic pathway of L-CARNITINE and is dependent on alpha-ketoglutarate; IRON; ASCORBIC ACID; and OXYGEN. 
History Note English:   2006(1980); use KETOGLUTARIC ACID 1973-1974, BETAINE 1972-1979, BUTYRATES 1974-1979, & AMINOBUTYRIC ACID 1970-1974 
Allowable Qualifiers English:  
AD administration & dosage AE adverse effects
AN analysis AI antagonists & inhibitors
BI biosynthesis BL blood
CF cerebrospinal fluid CS chemical synthesis
CH chemistry CL classification
CT contraindications DF deficiency
DE drug effects EC economics
GE genetics HI history
IM immunology IP isolation & purification
ME metabolism PK pharmacokinetics
PD pharmacology PH physiology
PO poisoning RE radiation effects
SE secretion ST standards
SD supply & distribution TU therapeutic use
TO toxicity UL ultrastructure
UR urine  
Record Number:   50661 
Unique Identifier:   D050563 

Occurrence in VHL: 		 

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