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Dicarboxylic Amino Acid Uptake in Normal, Friedreich's Ataxia, and Dicarboxylic Aminoaciduria Fibroblasts

Published online by Cambridge University Press:  18 September 2015

S.B. Melancon ,
B. Grenier ,
L. Dallaire ,
M. Potier ,
G. Fontaine ,
B. Grignon ,
G. Geoffroy ,
B. Lemieux  and
A. Barbeau
S.B. Melancon
Affiliation:
le Centre de Recherche Pédiatrique de l'Hôpital Ste-Justine, Montréal, l'Institut de Recherches Cliniques de Montréal, le Centre Hospitalier Universitaire de Sherbrooke
B. Grenier
Affiliation:
le Centre de Recherche Pédiatrique de l'Hôpital Ste-Justine, Montréal, l'Institut de Recherches Cliniques de Montréal, le Centre Hospitalier Universitaire de Sherbrooke
L. Dallaire
Affiliation:
le Centre de Recherche Pédiatrique de l'Hôpital Ste-Justine, Montréal, l'Institut de Recherches Cliniques de Montréal, le Centre Hospitalier Universitaire de Sherbrooke
M. Potier
Affiliation:
le Centre de Recherche Pédiatrique de l'Hôpital Ste-Justine, Montréal, l'Institut de Recherches Cliniques de Montréal, le Centre Hospitalier Universitaire de Sherbrooke
G. Fontaine
Affiliation:
le Centre de Recherche Pédiatrique de l'Hôpital Ste-Justine, Montréal, l'Institut de Recherches Cliniques de Montréal, le Centre Hospitalier Universitaire de Sherbrooke
B. Grignon
Affiliation:
le Centre de Recherche Pédiatrique de l'Hôpital Ste-Justine, Montréal, l'Institut de Recherches Cliniques de Montréal, le Centre Hospitalier Universitaire de Sherbrooke
G. Geoffroy
Affiliation:
le Centre de Recherche Pédiatrique de l'Hôpital Ste-Justine, Montréal, l'Institut de Recherches Cliniques de Montréal, le Centre Hospitalier Universitaire de Sherbrooke
B. Lemieux
Affiliation:
le Centre de Recherche Pédiatrique de l'Hôpital Ste-Justine, Montréal, l'Institut de Recherches Cliniques de Montréal, le Centre Hospitalier Universitaire de Sherbrooke
A. Barbeau
Affiliation:
le Centre de Recherche Pédiatrique de l'Hôpital Ste-Justine, Montréal, l'Institut de Recherches Cliniques de Montréal, le Centre Hospitalier Universitaire de Sherbrooke
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Glutamic and aspartic acid uptake was measured in skin fibroblasts from patients with Friedreich's Ataxia, dicarboxylic aminoaciduria, and normal individuals. The results showed no difference in uptake kinetics of either dicarboxylic amino acids between Friedreich's Ataxia and normal cells, but reduced uptake velocities in dicarboxylic aminoaciduria fibroblasts. Friedreich's Ataxia fibroblasts were, however, less calcium-dependant and more magnesium and phosphate-dependent than controls in glucose-free incubation mixture. This difference might be related to some degree of glucose intolerance by Friedreich's Ataxia fibroblasts in culture.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 6 , Issue 2 , May 1979 , pp. 263 - 273
Copyright
Copyright © Canadian Neurological Sciences Federation 1979

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