scholarly journals Effect of Asparagine, Glutamine and Insulin on Cerebral Amino Acid Neurotransmitters

1980 ◽  
Vol 7 (4) ◽  
pp. 447-450 ◽  
Author(s):  
Roger F. Butterworth ◽  
France Landreville ◽  
Edith Hamel ◽  
Andrea Merkel ◽  
François Giguere ◽  
...  
Keyword(s):  
Amino Acid ◽  
Medulla Oblongata ◽  
Neurological Disorders ◽  
Insulin Treatment ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Mammalian Brain ◽  
Amino Acid Neurotransmitters

SUMMARY:Treatment of rats with asparagine or glutamine caused substantial increases in glutamine concentrations in cerebellum and medulla oblongata. Insulin treatment caused a diminution of glutamate and GA BA in these regions of brain. Since it is now well-established that glutamine is a very efficient precursor of the neurotransmitter pool of glutamate in mammalian brain, treatment with asparagine or glutamine could be of therapeutic (replacement) value in the treatment of neurological disorders such as Friedreich's ataxia, in which cerebral glutamate concentrations have been found to be diminished.

scholarly journals Amino Acid Changes in Thiamine-Deficient Encephalopathy: Some Implications for the Pathogenesis of Friedreich's Ataxia

1979 ◽  
Vol 6 (2) ◽  
pp. 217-222 ◽  
Author(s):  
R.F. Butterworth ◽  
E. Hamel ◽  
F. Landreville ◽  
A. Barbeau
Keyword(s):  
Amino Acid ◽  
Pyruvate Decarboxylase ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Neuronal Dysfunction ◽  
Amino Acid Neurotransmitters ◽  
Important Amino Acid ◽  
Pyruvate Oxidation ◽  
Loss Of Righting Reflex ◽  
Righting Reflex

SummaryThiamine-deficient encephalopathy in the rat is characterized by ataxie gait, loss of righting reflex and curvature of the spine. N euro chemie ai changes include a diminished activity of cerebral pyruvate decarboxylase leading to abnormal pyruvate oxidation. The present study shows that this defective pyruvate oxidation produces a significant depletion of three important amino acid neurotransmitters, namely gamma aminobuiyrie acid (GABA), glutamic acid, andaspartic acid. Such changes could lead to severe neuronal dysfunction and the observed neurological symptoms of thiamine deficiency. Some implications for the pathogenesis of Friedreich's ataxia are discussed.

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

1979 ◽  
Vol 6 (2) ◽  
pp. 263-273 ◽  
Author(s):  
S.B. Melancon ◽  
B. Grenier ◽  
L. Dallaire ◽  
M. Potier ◽  
G. Fontaine ◽  
...  
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Amino Acid Uptake ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Acid Uptake ◽  
Normal Cells ◽  
Dicarboxylic Amino Acid ◽  
Normal Individuals ◽  
Kinetics Of

SummaryGlutamic 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.

scholarly journals Amino Acid Changes in the Mouse Mutant Dystonia Musculorum Similar to Those in Friedreich’s Ataxia

1982 ◽  
Vol 9 (2) ◽  
pp. 185-188 ◽  
Author(s):  
A. Messer
Keyword(s):  
Amino Acid ◽  
Experimental Model ◽  
Neurological Disorder ◽  
Autosomal Recessive ◽  
Red Nucleus ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Mouse Mutant ◽  
Sensory Afferents ◽  
Recessive Mutant

SUMMARY:An autosomal recessive mutant strain of mouse with a progressive neurological disorder is described. Histopathology is dramatic in the sensory afferents and in the red nucleus. In the cerebellar vermis the concentrations of glutamate, aspartate, glycine and GABA are significantly reduced, and in the cerebellar hemispheres the taurine/glutamate ratio is elevated. These mice may provide a useful experimental model of Friedreich’s ataxia.

scholarly journals Amino Acid Metabolism in Friedreich's Ataxia

1976 ◽  
Vol 3 (4) ◽  
pp. 373-378 ◽  
Author(s):  
B. Lemieux ◽  
A. Barbeau ◽  
V. Beroniade ◽  
D. Shapcott ◽  
G. Breton ◽  
...  
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Aspartic Acid ◽  
Plasma Levels ◽  
Genetic Defect ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Physiological Importance ◽  
And Control ◽  
Csf Concentration

SUMMARY:A study of amino acids determined by sequential Multi-sample Amino Acid Automatic Analyzer in plasma, urine and cerebrospinal fluid (CSF) in patients with Friedreich's ataxia and control subjects has revealed a number of mathematically significant variations from normal. Of practical physiological importance are the following: a high urinary excretion of alanine with slightly elevated plasma levels; a low plasma and CSF concentration of aspartic acid in the resence of normal urinary values and finally a low CSF concentration of taurine accompanied by normal plasma levels, but elevated urinary output and renal clearance rates. We postulate that the modifications in alanine and aspartic acid are less specific and probably secondary, but there could be a genetic defect in the membrane transport of taurine and the other β-amino acids in Friedreich's ataxia.

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

1979 ◽  
Vol 6 (2) ◽  
pp. 251-253 ◽  
Author(s):  
S.B. Melancon ◽  
B. Grignon ◽  
M. Potier ◽  
L. Dallaire
Keyword(s):  
Amino Acid ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Skin Fibroblasts ◽  
Carrier Protein ◽  
Human Skin Fibroblasts ◽  
Beta Alanine ◽  
Cultured Skin ◽  
Significant Difference ◽  
Amino Acid Carrier

SummaryTaurine 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.

scholarly journals Taurine in Cerebrospinal Fluid in Friedreich's Ataxia

1978 ◽  
Vol 5 (1) ◽  
pp. 125-129 ◽  
Author(s):  
B. Lemieux ◽  
R. Giguere ◽  
A. Barbeau ◽  
S. Melancon ◽  
D. Shapcott
Keyword(s):  
Amino Acids ◽  
Cerebrospinal Fluid ◽  
Amino Acid ◽  
Aspartic Acid ◽  
Amino Acid Analysis ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia

SUMMARY:In a previous study we reported low values of taurine and aspartic acid in the CSF of patients with Friedreich's ataxia, when the results were compared to the literature. Further studies have revealed that tinforetold difficulties with the advertised methodology of sequential multi-sample amino acid analysis were responsible for low values in the determination of these two amino acids in the small volumes necessary for CSF. A corrected method is presented. With the latter method the differences disappear for CSF taurine and aspartic acid, but they remain valid for the previously reported blood and urine values in Friedreich's ataxia. GABA levels are also normal in Friedreich's ataxia CSF.

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

1980 ◽  
Vol 7 (4) ◽  
pp. 441-446 ◽  
Author(s):  
S.B. Melançon ◽  
B. Grignon ◽  
E. Ledru ◽  
G. Geoffroy ◽  
M. Potier ◽  
...  
Keyword(s):  
Amino Acid ◽  
Transport System ◽  
Amino Acid Transport ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Skin Fibroblasts ◽  
Acid Uptake ◽  
Acid Transport ◽  
Amino Acid Transport System ◽  
Cultured Skin

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.

Otoneurological Findings in Friedreich's Ataxia and Other Inherited Neuropathies

1986 ◽  
Vol 25 (2) ◽  
pp. 84-91 ◽  
Author(s):  
E. Cassandro ◽  
F. Mosca ◽  
L. Sequino ◽  
F. A. De Falco ◽  
G. Campanella
Keyword(s):  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Inherited Neuropathies
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