The Beta-Amino Acid Transport System in Friedreich's Ataxia

Author:

Melançon S.B.,Grignon B.,Ledru E.,Geoffroy G.,Potier M.,Dallaire L.,Vanasse M.

Abstract

SUMMARY:Taurine and β-amino uptake in cultured skin fibroblasts proceeds through at least two distinct amino acid transport systems. The predominant Bamino acid uptake system which we refer to as the “Beta” system, incorporates taurine in a proportion of 95%, β-alanine in a proportion of 80% and does not incorporate β-amino-isobutyric acid. A second transport system for β-alanine seems to be operative in cultured skin fibroblasts and this system shares the characteristics of system “L” for branched-chain and ringside neutral amino acids. Results of ion depletion experiments, metabolic inhibition by drugs and blocking agents and previous kinetic studies of taurine and β-alanine uptake in cultured skin fibroblasts failed to disclose any major difference in β-amino acid transport between control individuals and patients with Friedreich's ataxia.

Publisher

Cambridge University Press (CUP)

Subject

Clinical Neurology,Neurology,General Medicine

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1. Growth Studies on Fibroblasts of Patients with Autosomal Recessive Friedreich’s Ataxia;Pathobiology;1991

2. Taurine uptake by normal and cystic fibrosis fibroblasts;Biochemistry and Cell Biology;1988-07-01

3. The inherited ataxias;Pediatric Neurology;1985-05

4. The Inherited Ataxias;Neurologic Clinics;1985-02

5. Platelet taurine content in Friedreich's disease;Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques;1984-11

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