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J R Soc Med. 1993 September; 86(9): 501–505.
PMCID: PMC1294093

Abnormal glycine metabolism in motor neurone disease: studies on plasma and cerebrospinal fluid.


Plasma amino acid levels were measured following oral glycine loading in 43 patients with motor neurone disease (MND), eight normal subjects and 18 neurological disease controls with wasting or spasticity from a variety of other causes. Levels at baseline and 1.5 h after loading did not differ, but at 4 h, plasma glycine levels in MND patients remained significantly higher than in normal and neurological controls (P < 0.013). Cerebrospinal fluid glycine levels, which were maximal at 2.5 h, were also significantly higher in MND patients than neurological controls (P < 0.04). These observations suggest a defect of glycine 'housekeeping' in the central nervous system in MND which may be relevant to the pathogenesis of the disease.

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Selected References

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  • Bank WJ, Morrow G., 3rd A familial spinal cord disorder with hyperglycinemia. Arch Neurol. 1972 Aug;27(2):136–144. [PubMed]
  • Bank WJ, Pizer L, Pfendner W. Glycine metabolism and spinal cord disorders. Adv Neurol. 1978;21:267–278. [PubMed]
  • de Belleroche J, Recordati A, Rose FC. Elevated levels of amino acids in the CSF of motor neuron disease patients. Neurochem Pathol. 1984 Spring;2(1):1–6. [PubMed]
  • Patten BM, Harati Y, Acosta L, Jung SS, Felmus MT. Free amino acid levels in amyotrophic lateral sclerosis. Ann Neurol. 1978 Apr;3(4):305–309. [PubMed]
  • Beal MF, Richardson EP., Jr Primary lateral sclerosis: a case report. Arch Neurol. 1981 Oct;38(10):630–633. [PubMed]
  • Gastaut JL, Michel B, Figarella-Branger D, Somma-Mauvais H. Chronic progressive spinobulbar spasticity. A rare form of primary lateral sclerosis. Arch Neurol. 1988 May;45(5):509–513. [PubMed]
  • Perry TL, Krieger C, Hansen S, Eisen A. Amyotrophic lateral sclerosis: amino acid levels in plasma and cerebrospinal fluid. Ann Neurol. 1990 Jul;28(1):12–17. [PubMed]
  • Plaitakis A, Caroscio JT. Abnormal glutamate metabolism in amyotrophic lateral sclerosis. Ann Neurol. 1987 Nov;22(5):575–579. [PubMed]
  • Schwarcz R, Meldrum B. Excitatory aminoacid antagonists provide a therapeutic approach to neurological disorders. Lancet. 1985 Jul 20;2(8447):140–143. [PubMed]
  • Spencer PS, Nunn PB, Hugon J, Ludolph AC, Ross SM, Roy DN, Robertson RC. Guam amyotrophic lateral sclerosis-parkinsonism-dementia linked to a plant excitant neurotoxin. Science. 1987 Jul 31;237(4814):517–522. [PubMed]
  • Spencer PS, Roy DN, Ludolph A, Hugon J, Dwivedi MP, Schaumburg HH. Lathyrism: evidence for role of the neuroexcitatory aminoacid BOAA. Lancet. 1986 Nov 8;2(8515):1066–1067. [PubMed]
  • Teitelbaum JS, Zatorre RJ, Carpenter S, Gendron D, Evans AC, Gjedde A, Cashman NR. Neurologic sequelae of domoic acid intoxication due to the ingestion of contaminated mussels. N Engl J Med. 1990 Jun 21;322(25):1781–1787. [PubMed]
  • Thomson AM. Glycine modulation of the NMDA receptor/channel complex. Trends Neurosci. 1989 Sep;12(9):349–353. [PubMed]
  • Johnson JW, Ascher P. Glycine potentiates the NMDA response in cultured mouse brain neurons. Nature. 1987 Feb 5;325(6104):529–531. [PubMed]
  • Plaitakis A. Glutamate dysfunction and selective motor neuron degeneration in amyotrophic lateral sclerosis: a hypothesis. Ann Neurol. 1990 Jul;28(1):3–8. [PubMed]
  • Lane RJ, Dick JP, de Belleroche J. Glycine and neurodegenerative disease. Lancet. 1991 Mar 23;337(8743):732–733. [PubMed]
  • Berger SJ, Carter JC, Lowry OH. The distribution of glycine, GABA, glutamate and aspartate in rabbit spinal cord, cerebellum and hippocampus. J Neurochem. 1977 Jan;28(1):149–158. [PubMed]
  • Shank RP, Aprison MH. The metabolism in vivo of glycine and serine in eight areas of the rat central nervous system. J Neurochem. 1970 Oct;17(10):1461–1475. [PubMed]
  • Logan WJ, Snyder SH. Unique high affinity uptake systems for glycine, glutamic and aspartic acids in central nervous tissue of the rat. Nature. 1971 Dec 3;234(5327):297–299. [PubMed]
  • Malessa S, Leigh PN, Bertel O, Sluga E, Hornykiewicz O. Amyotrophic lateral sclerosis: glutamate dehydrogenase and transmitter amino acids in the spinal cord. J Neurol Neurosurg Psychiatry. 1991 Nov;54(11):984–988. [PMC free article] [PubMed]
  • Dubowitz MN, Hughes PJ, Lane RJ, Wade JP. Gold-induced neuroencephalopathy responding to dimercaprol. Lancet. 1991 Apr 6;337(8745):850–851. [PubMed]
  • Rothstein JD, Martin LJ, Kuncl RW. Decreased glutamate transport by the brain and spinal cord in amyotrophic lateral sclerosis. N Engl J Med. 1992 May 28;326(22):1464–1468. [PubMed]

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