[Lysosomal enzyme deficiency: in vitro correlation of deficient cells using a conditioned medium or by co-culturing with non-deficient cells]. / Déficience en enzyme lysosomiale: correction in vitro de cellules déficitaires par du milieu conditionné ou par des co-cultures avec des cellules non déficitaires.
Pathol Biol (Paris)
; 37(1): 35-8, 1989 Jan.
Article
em Fr
| MEDLINE
| ID: mdl-2648267
ABSTRACT
We demonstrate that lysosomal enzyme (alpha-L-fucosidase) can enrich deficient fibroblasts, with purified enzyme brought by the medium, or with an enzyme supply by various cell sources. The co-culture systems lead to a deficient cell correction, whatever donor cells are lymphocytes or lymphoblastoid cells. This correction arise only with alive cells, and is strongly inhibited by mannose-6-phosphate. Our results do not support the hypothesis that cell to cell contact independently of mannose-6-phosphate binding site is necessary for transfer of lysosomal enzyme from lymphocytes to fibroblasts. We suggest that the neighbourhood of cells leads to a phosphorylated precursor increase in the pericellular area, which creates an enzyme stabilizing effect favourable at its incorporation.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Fibroblastos
/
Alfa-L-Fucosidase
Limite:
Humans
Idioma:
Fr
Revista:
Pathol Biol (Paris)
Ano de publicação:
1989
Tipo de documento:
Article
País de afiliação:
França