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Ornithine
(redirected from Ornithine carbamoyltransferase)

   Also found in: Dictionary/thesaurus, Medical, Acronyms, Wikipedia 0.01 sec.
ornithine [′ȯr·nə‚thēn]
(biochemistry)
C5H12O2N2An amino acid occurring in the urine of some birds, but not found in native proteins.

Ornithine 

H2N(CH2)3CH(NH2)COOH (also α, δ-di-aminovaleric acid), an amino acid that is readily soluble in water and alcohol.

Ornithine has one asymmetric carbon atom and exists in two optically active forms (D- and L-) and one racemic form, L-ornithine, found in the tissues of animals and humans, plays an important role in the biosynthesis of urea by the ornithine cycle. It is formed from arginine and α-amino-y-formylbutyric acid and may be converted to proline. Free L-ornithine is found in plants; bound L-ornithine is a component of several peptide antibiotics (bacitracin and gramicidin). L-ornithine has not been isolated from natural proteins.



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This dramatic case stumped physicians until the woman's blood revealed a telltale high level of ammonia, a diagnostic clue suggesting a rare, inherited disorder called ornithine carbamoyltransferase deficiency.
 
 
 
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