Cell-based therapy for RDEB: how does it work?
J Invest Dermatol
; 131(8): 1597-9, 2011 Aug.
Article
en En
| MEDLINE
| ID: mdl-21753764
No effective or specific treatment is currently available for recessive dystrophic epidermolysis bullosa (RDEB), a severe heritable blistering disorder caused by mutations in the type VII collagen gene (COL7A1). Recent studies have suggested that delivery of allogeneic fibroblasts to the skin of patients with RDEB may be beneficial in improving skin adhesion and increasing type VII collagen deposition at the dermal-epidermal junction. In this issue, Nagy et al. explore mechanisms of fibroblast therapy in a patient with RDEB displaying reduced type VII collagen protein expression at the dermal-epidermal junction. The results suggest that allogeneic fibroblast injection elicits expression of cytokines, including heparin binding-EGF-like growth factor (HB-EGF), that upregulate the expression of a patient-specific COL7A1 allele. Thus, fibroblast therapy may provide a novel way to improve skin therapy in a select subgroup of patients with this currently intractable disease.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Epidermólisis Ampollosa Distrófica
/
Trasplante de Médula Ósea
/
Trasplante de Células
/
Fibroblastos
Límite:
Humans
Idioma:
En
Revista:
J Invest Dermatol
Año:
2011
Tipo del documento:
Article
País de afiliación:
Estados Unidos
Pais de publicación:
Estados Unidos