[Lysosomal enzyme deficiency: in vitro correlation of deficient cells using a conditioned medium or by co-culturing with non-deficient cells].
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.
Veyron
P
P
Unité de Recherche en Néphro-Urologie Transplantation et Immunologie Clinique, INSERM U 80, Lyon, France.
el Habib
R
R
Zabot
M T
MT
Maire
I
I
Mathieu
M
M
Touraine
J L
JL
D002450:Cell Communication; D002478:Cells, Cultured; D003470:Culture Media; D005347:Fibroblasts / Q000201:enzymology*; D005645:Fucosidosis / Q000628:therapy; D006801:Humans; D005644:alpha-L-Fucosidase / Q000172:deficiency*
D003470:Culture Media; D005644:alpha-L-Fucosidase
Déficience en enzyme lysosomiale: correction in vitro de cellules déficitaires par du milieu conditionné ou par des co-cultures avec des cellules non déficitaires.