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gamma-Butyrobetaine Dioxygenase

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Definition: 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.      Other names gamma Butyrobetaine,2 Oxoglutarate Dioxygenase; gamma Butyrobetaine, 2 Oxoglutarate Dioxygenase; gamma Butyrobetaine Hydroxylase; gamma Butyrobetaine Dioxygenase; Hydroxylase, gamma-Butyrobetaine; Hydroxylase, Trimethylaminobutyrate; Hydroxylase, Butyrobetaine; Dioxygenase, gamma-Butyrobetaine,2-Oxoglutarate; Dioxygenase, gamma-Butyrobetaine; Dioxygenase gamma-Butyrobetaine, 2-Oxoglutarate; 2-Oxoglutarate Dioxygenase gamma-Butyrobetaine; gamma-Butyrobetaine,2-Oxoglutarate Dioxygenase; gamma-Butyrobetaine, 2-Oxoglutarate Dioxygenase; gamma-Butyrobetaine Hydroxylase; Trimethylaminobutyrate Hydroxylase; Butyrobetaine Hydroxylase; 4-Trimethylaminobutyrate, 2-Oxoglutarate Oxygen Ox
 
SubstanceCAS Registry & nameCategoriesSourceDrugs*
4-trimethylaminobutyric acid dioxygenase, mouse  EC 1.14.11.1   *gamma-Butyrobetaine Dioxygenase. Anal Biochem. 2006 Jul 1;354(1):132-9

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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