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Antioxidants inhibit SAA formation and pro-inflammatory cytokine release in a human cell model of alkaptonuria.


ABSTRACT:

Objective

Alkaptonuria (AKU) is an ultra-rare autosomal recessive disease that currently lacks an appropriate therapy. Recently we provided experimental evidence that AKU is a secondary serum amyloid A (SAA)-based amyloidosis. The aim of the present work was to evaluate the use of antioxidants to inhibit SAA amyloid and pro-inflammatory cytokine release in AKU.

Methods

We adopted a human chondrocytic cell AKU model to evaluate the anti-amyloid capacity of a set of antioxidants that had previously been shown to counteract ochronosis in a serum AKU model. Amyloid presence was evaluated by Congo red staining. Homogentisic acid-induced SAA production and pro-inflammatory cytokine release (overexpressed in AKU patients) were evaluated by ELISA and multiplex systems, respectively.

SUBMITTER: Spreafico A 

PROVIDER: S-EPMC3741479 | biostudies-literature | 2013 Sep

REPOSITORIES: biostudies-literature