Please use this identifier to cite or link to this item: http://hdl.handle.net/20.500.12779/6705
Title: Cytoskeleton Aberrations in Alkaptonuric Chondrocytes
Authors: Geminiani, Michela 
Gambassi, Silvia
Millucci, Lia 
Lupetti, Pietro
Collodel, Giulia
Mazzi, Lucia
Frediani, Bruno
Braconi, Daniela 
Mazzocchi, Barbara
Laschi, Marcella
Bernardini, Giulia 
Santucci, Annalisa 
Keywords: Actin; Alkaptonuria; Co-localization; SAA; Tubulin
Issue Date: 2016
Project: None 
Journal: JOURNAL OF CELLULAR PHYSIOLOGY
Abstract: 
Alkaptonuria (AKU) is an ultra-rare autosomal genetic disorder caused by a defect in the activity of the enzyme homogentisate 1,2-dioxygenase (HGD) that leads to the accumulation of homogentisic acid (HGA) and its oxidized product, benzoquinone acetic acid (BQA), in the connective tissues causing a pigmentation called "ochronosis". The consequent progressive formation of ochronotic aggregates generate a severe condition of oxidative stress and inflammation in all the affected areas. Experimental evidences have also proved the presence of serum amyloid A (SAA) in several AKU tissues and it allowed classifying AKU as a secondary amyloidosis. Although AKU is a multisystemic disease, the most affected system is the osteoarticular one and articular cartilage is the most damaged tissue. In this work, we have analyzed for the first time the cytoskeleton of AKU chondrocytes by means of immunofluorescence staining. We have shown the presence of SAA within AKU chondrocytes and finally we have demonstrated the co-localization of SAA with three cytoskeletal proteins: actin, vimentin and β-tubulin. Furthermore, in order to observe the ultrastructural features of AKU chondrocytes we have performed TEM analysis, focusing on the Golgi apparatus structure and, to demonstrate that pigmented areas in AKU cartilage are correspondent to areas of oxidation, 4-HNE presence has been evaluated by means of immunofluorescence. This article is protected by copyright. All rights reserved.
Description: 
101182
URI: http://hdl.handle.net/20.500.12779/6705
ISSN: 0021-9541
DOI: 10.1002/jcp.25500
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