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Titlebook: JIMD Reports, Volume 24; Johannes Zschocke,Matthias Baumgartner,Verena Pete Book 2015 SSIEM and Springer-Verlag Berlin Heidelberg 2015 Men

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Relationship Between Serum Concentrations of Nitisinone and Its Effect on Homogentisic Acid and Tyr). Nitisinone decreases HGA in AKU, but the concentration–response relationship has not been previously reported...: To determine the relationship between serum concentrations of nitisinone and the effect on both HGA and tyrosine; secondly to determine steady-state pharmacokinetics of nitisinone in
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Investigating the Robustness and Diagnostic Potential of Extracellular Matrix Remodelling Biomarkerding trial for nitisinone treatment of AKU patients. We tested a panel of serum and urinary biomarkers reflecting extracellular matrix remodelling (ECMR) of cartilage, bone and connective tissue in SONIA 1 patients to identify non-invasive and diagnostic biomarkers of tissue turnover in AKU...: Fast
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Nitisinone Arrests but Does Not Reverse Ochronosis in Alkaptonuric Mice, in the catabolism of phenylalanine and tyrosine. Loss of HGD function prevents metabolism of homogentisic acid (HGA), leading to increased levels of plasma HGA and urinary excretion. Excess HGA becomes deposited in collagenous tissues and subsequently undergoes polymerisation, principally in the ca
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The Pigment in Alkaptonuria Relationship to Melanin and Other Coloured Substances: A Review of Metaain pigment melanin in defining its biochemistry, the different types and function. However, analytical techniques to show these differences in vivo are still not readily available. NMR and IR spectroscopy are relatively insensitive and reveal only major structural differences. Techniques utilising
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