Please use this identifier to cite or link to this item: http://hdl.handle.net/20.500.12779/5813
Title: Evaluation of anti-oxidant treatments in an in vitro model of alkaptonuric ochronosis
Authors: Braconi, Daniela 
Laschi, Marcella
Amato, Loredana
Bernardini, Giulia 
Millucci, Lia 
Marcolongo, R
Cavallo, G
Spreafico, Adriano
Santucci, Annalisa 
Issue Date: 2010
Project: None 
Journal: RHEUMATOLOGY
Abstract: 
OBJECTIVES: Alkaptonuria (AKU) is a rare genetic disease associated with deficient homogentisate 1,2-dioxygenase activity in the liver. This leads to the accumulation of homogentisic acid (HGA) and its oxidized/polymerized products in connective tissues, which in turn become characterized by the presence of melanin-like pigments (ochronosis). Since at present, further studies are necessary to support the use of drugs for the treatment of AKU, we investigated the effects of various anti-oxidants in counteracting melanin-like pigmentation and oxidative stress related to HGA and its metabolites. METHODS: We set up an in vitro model using human serum treated with 0.33 mM HGA and tested the anti-oxidants ascorbic acid, N-acetylcysteine, phytic acid (PHY), taurine (TAU), ferulic acid (FER) and lipoic acid (LIP) for their ability to prevent or delay the production of melanin-like pigments, as well as to reduce oxidative post-translational modifications of proteins. Monitoring of intrinsic fluorescence of HGA-induced melanin-like pigments was used to evaluate the efficacy of compounds. RESULTS: Our model allowed us to prove efficacy especially for PHY, TAU, LIP and FER in counteracting the production of HGA-induced melanin-like pigments and protein oxidation induced by HGA and its metabolites. CONCLUSIONS: Our model allows the opening of new anti-oxidant therapeutic strategies to treat alkaptonuric ochronosis.
Description: 
24597
URI: http://hdl.handle.net/20.500.12779/5813
ISSN: 1462-0324
DOI: 10.1093/rheumatology/keq175
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