Dealba Gheduzzi (Avtor), Federica Boraldi (Avtor), Giulia Annovi (Avtor), Chiara Paolinelli DeVincenzi (Avtor), Leon J. Schurgers (Avtor), Cees Vermeer (Avtor), Daniela Quaglino (Avtor), Ivonne Pasquali Ronchetti (Avtor)

Povzetek

Mature MGP (Matrix g-carboxyglutamic acid protein) is known to inhibit soft connective tissues calcification. We investigated its possible involvement in pseudoxanthoma elasticum (PXE), a genetic disorder whose clinical manifestations are due to mineralization of elastic fibers. PXE patients have lower serum concentration of total MGP compared to controls (Po0.001). Antibodies specific for the noncarboxylated (Glu-MGP) and for the g-carboxylated (Gla-MGP) forms of MGP were assayed on ultrathin sections of dermis from controls and PXE patients. Normal elastic fibers in controls and patients were slightly positive for both forms of MGP, whereas Gla-MGP was more abundant within control’s than within patient’s elastic fibers (Po0.001). In patients’ calcified elastic fibers, Glu-MGP intensively colocalized with mineral precipitates, whereas Gla-MGP precisely localized at the mineralization front. Data suggest that MGP is present within elastic fibers and is associated with calcification of dermal elastic fibers in PXE.

Ključne besede

calcification;dermal fibroblast;elastic fiber;human skin;MGP;pseudoxanthoma elasticum;

Podatki

Jezik: Angleški jezik
Leto izida:
Tipologija: 1.01 - Izvirni znanstveni članek
Organizacija: UNG - Univerza v Novi Gorici
UDK: 616
COBISS: 5424635 Povezava se bo odprla v novem oknu
ISSN: 0023-6837
Št. ogledov: 2586
Št. prenosov: 0
Ocena: 0 (0 glasov)
Metapodatki: JSON JSON-RDF JSON-LD TURTLE N-TRIPLES XML RDFA MICRODATA DC-XML DC-RDF RDF

Ostali podatki

URN: URN:SI:UNG
Vrsta dela (COBISS): Delo ni kategorizirano
Strani: str. 998-1008
Letnik: ǂVol. ǂ87
Zvezek: ǂiss. ǂ10
Čas izdaje: Oct. 2007
DOI: 10.1038/labinvest.3700667
ID: 11192838