Vascular Endothelial Growth Factor (VEGF-a) in Fabry disease: Association with cutaneous and systemic manifestations with vascular involvement

Chiara Cadeddu, Anna Zampetti, Chiara De Waure, Claudio Feliciani, Walter Borsini, Maurizio Pieroni

Risultato della ricerca: Contributo in rivistaArticolo in rivista

10 Citazioni (Scopus)

Abstract

Fabry disease is an X-linked inherited metabolic disorder characterized by the deficiency of lysosomal α-galactosidase A enzyme. This leads to the accumulation, into lysosomes through the body, of glycosphingolipids, mainly Gb3. Skin involvement and progressive multi-organ failure are usually observed. Endothelium is the preferential target of the Gb3 storage that determines endothelial dysfunction and vasculopathy leading to the clinical manifestations of the disease. The serum levels of Vascular Endothelial Growth Factor-A (VEGF-A), a specific endothelial cell mitogen, were analyzed in Fabry patients to explore a possible association to the clinical manifestations with vascular involvement.
Lingua originaleEnglish
pagine (da-a)933-939
Numero di pagine7
RivistaCytokine
Volume61
DOI
Stato di pubblicazionePubblicato - 2013

Keywords

  • fabry
  • vegf

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