Background. Complex polysaccharides, glycosaminoglycans (GAGs), their amount and fine structure were determined in the skin (epidermis + dermis) of pseudoxanthoma elasticum (PXE)-affected patients in comparison with healthy subjects. Methods. Non-lesional skin GAGs were extracted and specifically determined by enzymatic treatment and HPLC separation.Results. Dermatan sulfate (DS) and hyaluronic acid (HA) were found the major GAG species with a DS percentage of approx. 20, a HA content of 58% and a chondroitin sulfate (CS) unsaturated 6-sulfated disaccharide amount of about 21%. Skin from PXE patients showed a similar HA percentage (61%), a corresponding DS content (22%) and no modification of the CS 6-sulfated disaccharide (16.7%). No changes of the total charge density and non-sulfated/sulfated GAGs ratio was noted along with no modification of the position of the sulfate groups (4s/6s) on the CS/DS backbone for PXE-affected subjects. However, a significant increase by about 88% (p<0.01) of the total amount of GAGs (HA+DS+CS) was found in PXE groups versus normal subjects. Conclusions. The altered metabolic processes produce in the skin of PXE-affected patients an increase in the total GAGs able to accumulate salts, in particular calcium ions, within the elastic fibers and to produce ion precipitates affecting the organization of matrix fibre.
Structural characterization of the skin glycosaminoglycans in patients with pseudoxanthoma elasticum / Volpi, Nicola; Maccari, Francesca. - In: INTERNATIONAL JOURNAL OF DERMATOLOGY. - ISSN 0011-9059. - STAMPA. - 47:10(2008), pp. 1024-1027. [10.1111/j.1365-4632.2008.03762.x]
Structural characterization of the skin glycosaminoglycans in patients with pseudoxanthoma elasticum
VOLPI, Nicola;MACCARI, Francesca
2008
Abstract
Background. Complex polysaccharides, glycosaminoglycans (GAGs), their amount and fine structure were determined in the skin (epidermis + dermis) of pseudoxanthoma elasticum (PXE)-affected patients in comparison with healthy subjects. Methods. Non-lesional skin GAGs were extracted and specifically determined by enzymatic treatment and HPLC separation.Results. Dermatan sulfate (DS) and hyaluronic acid (HA) were found the major GAG species with a DS percentage of approx. 20, a HA content of 58% and a chondroitin sulfate (CS) unsaturated 6-sulfated disaccharide amount of about 21%. Skin from PXE patients showed a similar HA percentage (61%), a corresponding DS content (22%) and no modification of the CS 6-sulfated disaccharide (16.7%). No changes of the total charge density and non-sulfated/sulfated GAGs ratio was noted along with no modification of the position of the sulfate groups (4s/6s) on the CS/DS backbone for PXE-affected subjects. However, a significant increase by about 88% (p<0.01) of the total amount of GAGs (HA+DS+CS) was found in PXE groups versus normal subjects. Conclusions. The altered metabolic processes produce in the skin of PXE-affected patients an increase in the total GAGs able to accumulate salts, in particular calcium ions, within the elastic fibers and to produce ion precipitates affecting the organization of matrix fibre.File | Dimensione | Formato | |
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