Accession NumberHMDB02354
Common_NameHawkinsin
DescriptionHawkinsin is a sulfur amino acid identified as (2-l-cystein-S-yl, 4-dihydroxycyclohex-5-en-1-yl)acetic acid. Patients with hawkinsinuria excrete this metabolite in their urine throughout their life, although symptoms of metabolic acidosis and tyrosinemia improve in the first year of life. Alterations in the structure and activity of 4-hydroxyphenylpyruvic acid dioxygenase are causally related to two different metabolic disorders, tyrosinemia type III and hawkinsinuria. (PMID 11073718)
Chemical_IUPAC_Name2-amino-3-[[2-(carboxymethyl)-2,5-dihydroxy-1-cyclohex-3 enyl]sulfanyl]propanoic acid
Chemical FormulaC11H17NO6S
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Predicted 13C NMR PeaklistDownload
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