Please use this identifier to cite or link to this item: http://hdl.handle.net/20.500.12779/5771
DC FieldValueLanguage
dc.contributor.authorTinti, Len_us
dc.contributor.authorSpreafico, Aen_us
dc.contributor.authorBraconi, Danielaen_us
dc.contributor.authorMillucci, Liaen_us
dc.contributor.authorBernardini, Giuliaen_us
dc.contributor.authorChellini, Federicoen_us
dc.contributor.authorCavallo, Gen_us
dc.contributor.authorSelvi, Een_us
dc.contributor.authorGaleazzi, Mauroen_us
dc.contributor.authorMarcolongo, Ren_us
dc.contributor.authorGallagher, Jen_us
dc.contributor.authorSantucci, Annalisaen_us
dc.date.accessioned2021-03-30T15:55:01Z-
dc.date.available2021-03-30T15:55:01Z-
dc.date.issued2010-
dc.identifier.issn0021-9541en_US
dc.identifier.urihttp://hdl.handle.net/20.500.12779/5771-
dc.description37515en_US
dc.description.abstractAlkaptonuria (AKU) is a rare autosomal recessive disease, associated with deficiency of homogentisate 1,2-dioxygenase activity in the liver. This leads to an accumulation of homogentisic acid (HGA) and its oxidized derivatives in polymerized form in connective tissues especially in joints. Currently, AKU lacks an appropriate therapy. Hence, we propose a new treatment for AKU using the antioxidant N-acetylcysteine (NAC) administered in combinations with ascorbic acid (ASC) since it has been proven that NAC counteracts the side-effects of ASC. We established an in vitro cell model using human articular primary chondrocytes challenged with an excess of HGA (0.33 mM). We used this experimental model to undertake pre-clinical testing of potential antioxidative therapies for AKU, evaluating apoptosis, viability, proliferation, and metabolism of chondrocytes exposed to HGA and treated with NAC and ASC administered alone or in combination addition of both. NAC decreased apoptosis induced in chondrocytes by HGA, increased chondrocyte growth reduced by HGA, and partially restored proteoglycan release inhibited by HGA. A significantly improvement in efficacy was found with combined addition of the two antioxidants in comparison with NAC and ASC alone. Our novel in vitro AKU model allowed us to demonstrate the efficacy of the co-administration of NAC and ASC to counteract the negative effects of HGA for the treatment of ochronotic arthropathy.en_US
dc.language.isoenen_US
dc.relationNoneen_US
dc.relation.ispartofJOURNAL OF CELLULAR PHYSIOLOGYen_US
dc.titleEvaluation of antioxidant drugs for the treatment of ochronotic alkaptonuria in an in vitro human cell modelen_US
dc.typeArticleen_US
dc.identifier.doi10.1002/jcp.22199en_US
dc.identifier.pmid20648626en_US
dc.identifier.scopus2-s2.0-77956553614en_US
dc.identifier.isiWOS:000281295100010en_US
dc.identifier.urlhttp://onlinelibrary.wiley.com/doi/10.1002/jcp.22199/abstracten_US
dc.relation.volume225(1)en_US
dc.description.firstpage84en_US
dc.description.lastpage91en_US
dc.description.thirdmissionNot applicableen_US
item.cerifentitytypePublications-
item.grantfulltextnone-
item.languageiso639-1en-
item.openairecristypehttp://purl.org/coar/resource_type/c_18cf-
item.openairetypeArticle-
item.fulltextNo Fulltext-
crisitem.author.orcid0000-0002-9657-4169-
crisitem.author.orcid0000-0002-5379-8808-
crisitem.author.orcid0000-0003-0016-0728-
crisitem.author.orcid0000-0001-6976-9086-
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