Determinants of tyrosinaemia during nitisinone therapy in alkaptonuria.

Sci Rep · 2022 · PMC9515198 · PMID 36167967

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Combined metabolites represent the daily generation of metabolites in the liver and kidney, and shows convincingly significantly increased formation of TYR and HPLA from HPPA upon HPPD inhibition; the sHPPA/sTYR and cHPPA 24 /cTYR 24 as well as sHPPA/sHPLA and cHPPA 24 /cHPLA 24 decreased with increasing sTYR indicating that TYR and HPLA formation was greater than HPPA during NIT treatment.

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