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: 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:
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Authors | Daniela Braconi, Marcella Laschi, Loredana Amato, Giulia Bernardini, Lia Millucci, Roberto Marcolongo, Giovanni Cavallo, Adriano Spreafico, Annalisa Santucci |
Journal | Rheumatology (Oxford, England)
(Rheumatology (Oxford))
Vol. 49
Issue 10
Pg. 1975-83
(Oct 2010)
ISSN: 1462-0332 [Electronic] England |
PMID | 20601653
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Antioxidants
- Coumaric Acids
- Taurine
- Thioctic Acid
- Phytic Acid
- ferulic acid
- Homogentisic Acid
- Ascorbic Acid
- Acetylcysteine
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Topics |
- Acetylcysteine
(therapeutic use)
- Alkaptonuria
(drug therapy)
- Antioxidants
(therapeutic use)
- Ascorbic Acid
(therapeutic use)
- Cells, Cultured
- Coumaric Acids
(therapeutic use)
- Homogentisic Acid
(metabolism)
- Humans
- Ochronosis
(drug therapy)
- Oxidative Stress
(drug effects)
- Phytic Acid
(therapeutic use)
- Protein Carbonylation
(drug effects)
- Taurine
(therapeutic use)
- Thioctic Acid
(therapeutic use)
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