Skip to main content
SpringerLink
Log in

Plasma amino acids concentration in amyotrophic lateral sclerosis patients

  • Published:
Amino Acids Aims and scope Submit manuscript

Summary.

 Previous investigations showed an impairment of amino acids (AA) metabolism in amyotrophic lateral sclerosis (ALS). It was hypothesized that excitatory AA may play an important role in the etiopathogenesis of this disease. The aim of the study was to determine plasma AA concentrations in ALS patients, and to examine the relationship between AA and the clinical state of ALS patients, the type of ALS onset and the duration of the disease. The study involved 20 ALS patients and 30 control group people. The AA analysis was performed by ion – exchange chromatography on an automatic AA analyser. The results showed significantly decreased concentrations of valine, isoleucine, leucine, tyrosine and aspartate in the plasma of the whole group of ALS patients compared to the control group, and a significantly decreased concentration of arginine in the patients with a long duration of ALS compared to the patients with a short duration. The clinical state of ALS patients significantly influenced only plasma alanine concentration. Other plasma AA concentrations were not significantly associated with clinical parameters of the disease. Our study confirms that metabolic abnormalities concerning AA exist in ALS patients. However, the normal plasma glutamate concentration observed in this study in the whole group of ALS patients compared to the controls does not exclude that this excitatory AA may play a role in neurodegeneration in ALS.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  • Babu GN, Bawari M, Mathur VN, Kalita J, Misra UK (1998) Blood glutamate levels in patients with motor neuron disease. Clin Chim Acta 273: 195–200

    Article  CAS  PubMed  Google Scholar 

  • Bendotti C, Tortarolo M, Suchak SK, Calvaresi N, Carvelli L, Bastone A, Rizzi M, Rattray M, Mennini T (2001) Transgenic SOD1 G93A mice develop reduced GLT-1 in spinal cord without alterations in cerebrospinal fluid glutamate levels. J Neurochem 79: 737–746

    Article  CAS  PubMed  Google Scholar 

  • Blin O, Desnuelle C, Guelton C, Aubrespy G, Ardissone JP, Crevat A, Pouget J, Serratrice G (1991) Anomaly in the neurotransmitter amino acids in amyotrophic lateral sclerosis: a therapeutic application. Rev Neurol (Paris) 147: 392–394

    CAS  Google Scholar 

  • Brooks BR (1994) El Escorial Word Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. J Neurol Sci 124 [Suppl]: 96–107

    Article  PubMed  Google Scholar 

  • Camu W, Billiard M, Baldy–Moulinier M (1993) Fasting plasma and CSF amino acid levels in amyotrophic lateral sclerosis: a subtype analysis. Acta Neurol Scand 88: 51–55

    CAS  Google Scholar 

  • Cluskey S, Ramsden DB (2001) Mechanisms of neurodegeneration in amyotrophic lateral sclerosis. Mol Pathol 54: 386–392

    CAS  PubMed Central  PubMed  Google Scholar 

  • Desport JC, Preux PM, Truong TC, Vallat JM, Sautereau D, Couratier P (1999) Nutritional status is a prognostic factor for survival in ALS patients. Neurology 53: 1059–1063

    Article  CAS  PubMed  Google Scholar 

  • Desport JC, Preux PM, Truong CT, Courat L, Vallat JM, Couratier P (2000) Nutritional assessment and survival in ALS patients. Amyotroph Lateral Scler Other Motor Neuron Disord 1: 91– 96

    Google Scholar 

  • Dillon BJ, Holtsberg FW, Ensor CM, Bomalaski JS, Clark MA (2002) Biochemical characterization of the arginine degrading enzymes arginase and arginine deiminase and their effect on nitric oxide production. Med Sci Monit 8: 248–253

    Google Scholar 

  • Freund HR, Hanani M (2002) The metabolic role of branched – chain amino acids. Nutrition 18: 287–288

    Article  PubMed  Google Scholar 

  • Holecek M (2002) Relation between glutamine, branched – chain amino acids, and protein metabolism. Nutrition 18: 130–133 Iwasaki Y, Ikeda K, Kinoshita M (1992) Plasma amino acid levels in patients with amyotrophic lateral sclerosis. J Neurol Sci 107: 219– 222

    Google Scholar 

  • Kean EA (1967) The relationship between nutritive value of dietary protein and activity of liver arginase and kidney transaminidase enzymes. Br J Nutr 21: 29–36

    Article  CAS  PubMed  Google Scholar 

  • Lal H, Chugh K (1995) Metabolic and regulatory effects of branched chain amino acid supplementation. Nutrition Res 15: 1717–1733

    Article  CAS  Google Scholar 

  • Liu B, Gao HM, Wang JY, Jeohn GH, Cooper CL, Hong JS (2002) Role of nitric oxide in inflammation – mediated neurodegeneration. Ann NY Acad Sci 962: 318–331

    Article  CAS  PubMed  Google Scholar 

  • Mayer ML, Vyklicky L Jr, Clements J (1989) Regulation of NMDA receptor desensitization in mouse hippocampal neurons by glycine. Nature 338: 425–427

    Article  CAS  PubMed  Google Scholar 

  • Meier DH, Schott KJ (1988) Free amino acid pattern of cerebrospinal fluid in amyotrophic lateral sclerosis. Acta Neurol Scand 77: 50–53

    Article  CAS  PubMed  Google Scholar 

  • Meister A, Anderson ME (1983) Glutathione. Annu Rev Biochem 52: 711–760

    Article  CAS  Google Scholar 

  • Moore S, Spackman DD, Stein WH (1958) Chromatography of amino acids on sulfonated polystyrene resins. Ann Chem 30: 1185–1189

    Article  CAS  Google Scholar 

  • Mori M, Gotoh T (2000) Regulation of nitric oxide production by arginine metabolic enzymes. Biochem Biophys Res Commun 275: 715–719

    Article  CAS  PubMed  Google Scholar 

  • Niebroj–Dobosz I, Janik P (1999) Amino acids acting as transmitters in amyotrophic lateral sclerosis (ALS). Acta Neurol Scand 100: 6–11

    Google Scholar 

  • Patten BM, Harati Y, Acosta L, Jung SS, Felmus MT (1978) Free amino acid levels in amyotrophic lateral sclerosis. Ann Neurol 3: 305–309

    Article  CAS  PubMed  Google Scholar 

  • Perry TL, Krieger C, Hansen S, Eisen A (1990) Amyotrophic lateral sclerosis: amino acid levels in plasma and cerebrospinal fluid. Ann Neurol 28: 12–17

    Article  CAS  PubMed  Google Scholar 

  • Plaitakis A (1991) Altered glutamatergic mechanisms and selective motor neuron degeneration in amyotrophic lateral sclerosis: possible role for glycine. Adv Neurol 56: 319–336

    CAS  PubMed  Google Scholar 

  • Plaitakis A, Constantakakis E (1993) Altered metabolism of excitatory amino acids, N-acetyl-aspartate and N-acetyl-aspartylglutamate in amyotrophic lateral sclerosis. Brain Res Bull 30: 381–386

    Google Scholar 

  • Puka-Sundvall M, Eriksson P, Nilsson M, Sandberg M, Lehmann A (1995) Neurotoxicity of cysteine: interaction with glutamate. Brain Res 705: 65–70

    Article  CAS  PubMed  Google Scholar 

  • Riviere M, Meininger V, Zeisser P, Munsat T (1998) An analysis of extended survival in patients with amyotrophic lateral sclerosis treated with riluzole. Arch Neurol 55: 526–528

    Article  CAS  PubMed  Google Scholar 

  • Rothstein JD (1995) Excitotoxic mechanisms in the pathogenesis of amyotrophic lateral sclerosis. Adv Neurol 68: 7–20 lateral sclerosis. Ann Neurol 28: 18–25 Silani V, Kasarskis EJ, Yanagisawa N (1998) Nutritional management in amyotrophic lateral sclerosis: a worldwide perspective. J Neurol 245 [Suppl 2]: 13–19

    Google Scholar 

  • Spreux-Varoquaux O, Bensimon G, Lacomblez L, Salachas F, Pradat PF, Le Forestier N, Marouan A, Dib M, Meininger V (2002) Glutamate levels in cerebrospinal fluid in amyotrophic lateral sclerosis: a reappraisal using a new HPLC method with coulometric detection in a large cohort of patients. J Neurol Sci 193: 73–78

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1.  Department of Neurology, Medical University, Lublin, Poland, Poland

    J. Ilżecka & Z. Stelmasiak

  2.  Department of Clinical Analytics, Medical University, Lublin, Poland, Poland

    J. Solski

  3.  Department of Chemistry, Medical University, Lublin, Poland, Poland

    S. Wawrzycki & M. Szpetnar

Authors
  1. J. Ilżecka
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Z. Stelmasiak
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. J. Solski
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. S. Wawrzycki
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. M. Szpetnar
    View author publications

    You can also search for this author in PubMed Google Scholar

Additional information

Correspondence: Joanna Iłżecka M.D., Department of Neurology, Medical University, Jaczewskiego 8, 20-954 Lublin, Poland, Fax: +48 81 742 55 34, E-mail: Ilzecka@medscape.com

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ilżecka, J., Stelmasiak, Z., Solski, J. et al. Plasma amino acids concentration in amyotrophic lateral sclerosis patients. Amino Acids 25, 69–73 (2003). https://doi.org/10.1007/s00726-002-0352-2

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00726-002-0352-2

Search

Navigation