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Taurine and Beta-Alanine Uptake in Cultured Human Skin Fibroblasts from Patients with Friedreich's Ataxia

Published online by Cambridge University Press:  18 September 2015

S.B. Melancon ,
B. Grignon ,
M. Potier  and
L. Dallaire
S.B. Melancon
Affiliation:
le Centre de Recherche Pédiatrique de l'Hôpital Ste-Justine, Département de Pédiatrie, Université de Montréal
B. Grignon
Affiliation:
le Centre de Recherche Pédiatrique de l'Hôpital Ste-Justine, Département de Pédiatrie, Université de Montréal
M. Potier
Affiliation:
le Centre de Recherche Pédiatrique de l'Hôpital Ste-Justine, Département de Pédiatrie, Université de Montréal
L. Dallaire
Affiliation:
le Centre de Recherche Pédiatrique de l'Hôpital Ste-Justine, Département de Pédiatrie, Université de Montréal
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Summary

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Taurine and ß-alanine uptake kineiics were studied in cultured skin fibroblasts from 9 patients with Friedreich's Ataxia and 8 controls. No significant difference was observed. The data support the presence of normal ß-amino acid carrier protein in Friedreich's Ataxia cell membrane.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 6 , Issue 2 , May 1979 , pp. 251 - 253
Copyright
Copyright © Canadian Neurological Sciences Federation 1979

References

Barbeau, A. (1978) Friedreich's Ataxia 1978 — An Overview. Can. J. Neurol. Sci. 5: 161–165.CrossRefGoogle ScholarPubMed
Booth, C.W. and Nadler, H.L. (1975). Neutral amino acid transport in cultivated human skin fibroblasts. Proc. Soc. Exp. Biol. Med. 148, 277–282.CrossRefGoogle Scholar
Butterworth, R. F., Hamel, E., Lan-Dreville, F., and Barbeau, A. (1978a). Cerebellar ataxia produced by 3-acetyl pyridine in rats. Can. J. Neurol. Sci., 5, 131–134.CrossRefGoogle Scholar
Filla, A., Butterworth, R. F., Geoffroy, G., Lemieux, B. and Barbeau, A. (1978). Platelet taurine uptake in spinocerebellar degeneration. Can. J. Neurol. Sci., 5, 119–123.CrossRefGoogle ScholarPubMed
Groth, V. and Rosenberg, L. E. (1972). Transport of dibasic amino acids, cystine and tryptophan by cultured human fibroblasts: Absence of a defect in cystinuria and Hartnup disease. J. Clin. Invest., 51, 2130–2142.CrossRefGoogle ScholarPubMed
Hillman, R. E. and Otto, E.F. (1974). Transport of L-isoleucine by cultured human fibroblasts. J. Biol. Chem. 249, (11) 3430–3435.CrossRefGoogle ScholarPubMed
Kaye, C.I. and Nadler, H.L. (1976). Transport of L-cystine by cultivated skin fibrotülast of normal subjects and patients with cystinosis. Pediat. Res. 10, 637–641.CrossRefGoogle ScholarPubMed
Lemieux, B., Barbeau, A., Beron-Iade, V., Shapcott, D., Breton, G., Geoffroy, G. and Melancon, S. B. (1976). Amino acid metabolism in Fried-reich's Ataxia. Can. J. Neurol. Sci., 3, 373–378.CrossRefGoogle ScholarPubMed
Melancon, S. B., Grenier, B., Dall-Aire, L., Potier, M., Fontaine, G.Grignon, B., Geoffroy, G., Lemieux, B. and Barbeau, A. (1979). Dicarboxylic amino acid uptake in normal, Friedreich's Ataxia and dicarboxylic aminoaciduria fibroblasts. Can. J. Neurol. Sci., this issue.CrossRefGoogle ScholarPubMed
Pellefigue, F., Debrohun Butler, J.Spielberg, S. P., Hollenberg, M. D.Goodman, S. I. and Schulman, J. D.Normal amino acid uptake by cultured human fibroblasts does notsrequire gammaglutamyl transpeptidase. Biochem. Biophys. Res. Comm. 73, 4: 997–1002.Google Scholar
Revsin, B. and Morrow, G. (1976). Glycine transport in normal and non-ketotic hyperglycinemic human diploid fibroblasts. Exptl. Cell Res. 100, 95–103.CrossRefGoogle ScholarPubMed
Ullrich, J. and Fromter, E. (1978). Active and passive transtubular transport in the proximal convolution. Proceed VII th Int. Congr. Nephrol. Montreal, Ed. R. Barceloet al. pp. 147–154.Google Scholar