Background: Down syndrome (DS) is a genetic disorder (trisomy 21 in 96% of cases), associated with an excess of a key enzyme involved with free radical metabolism (FRM), superoxide dismutase-1 (SOD-1), that is encoded by a gene on chromosome 21. Consequently, SOD-1 activity is elevated in DS, which also occurs in conditions of oxidative stress, and is associated with a compensatory increase in glutathione peroxidase activity (GSHPx).
Methods: This study examined the relationship of memory function with erythrocyte SOD-1, GSHPx and catalase (CAT) activity in 22–51 year old adults with DS.
Results: Mean erythrocyte SOD-1 (p < .02) and GSHPx (p < .01), but not CAT (p = .76), activities were significantly greater in the DS group than the controls. In the DS group, erythrocyte GSHPx, but not SOD-1 or CAT activities, was significantly correlated with memory function (r = .625, p < .025, df = 13 for savings score, r = .631, p < .01, df = 14 for intrusion errors) but not with intelligence quotients.
Conclusions: These observations suggest a possible relationship between altered FRM with memory deficits among adults with DS within the age-range in that an age-related increase in the prevalence for Alzheimer’s neuropathology is known to be robust before reaching a plateau of 100%.
To read this article in full you will need to make a payment
Purchase one-time access:Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
One-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:Subscribe to Biological Psychiatry
Already a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
- Lipid peroxidation and superoxide dismutase-1 and glutathione peroxidase activities in trisomy 21 fetal mice and human trisomy 21 fibroblasts.Pediatr Res. 1987; 21: 88-92
- Red Cell Metabolism. 3rd ed. Grune & Stratton, Orlando, FL1984
- Superoxide dismutase, glutathione peroxidase and lipoperoxidation in Down’s syndrome fetal brain.Dev Brain Res. 1984; 16: 37-44
- Serological alpha-1 antichymotrypsin in Down’s syndrome and Alzheimer’s disease.Ann Neurol. 1992; 32: 193-197
- Evidence for accelerated skin wrinkling among developmentally delayed individuals with Down’s syndrome.Mech Aging Dev. 1993; 70: 213-225
- Cognitive impairment in adults with Down’s syndrome.Neurology. 1994; 44: 232-238
- Differentiation of amnesiac and demented patients with the Wechsler Memory Scale-Revised.Clin Neuropsychol. 1999; 2: 133-148
- Expression of transfected human Cu-Zn superoxide dismutase gene in mouse L cells and NS20Y neuroblastoma cells induces enhancement of glutathione peroxidase activity.Biochem Biophys Acta. 1988; 949: 58-64
- Expression of human Cu-Zn superoxide dismutase gene in transgenic mice.Free Radic Res Commun. 1991; 12: 581-589
- Neuronal-specific expression of human Cu-Zn superoxide dismutase gene in transgenic mice.Brain Res. 1991; 552: 198-214
- Cognitive and functional status of the oldest old.J Am Geri Soc. 1996; 44: 671-674
- Association between Alzheimer’s Disease and Down’s Syndrome. Oxford University Press, London1992
- Profiles of demented and amnesiac patients on the California Learning Test.Psychol Assess: J Consult Clin Psychol. 1991; 3: 19-26
- Normal aging in adults with Down’s syndrome.J Intel Dis Res. 1996; 40: 208-221
- A rapid one-step procedure for purification of mononuclear and polymorphonuclear leukocytes from human bleed using a modification of the hypaque-ficoll technique.J Immunol Meth. 1978; 24: 389-393
- Superoxide dismutase in Down syndrome.Lancet. 1975; ii: 42-43
- Down syndrome clinical symptoms are manifested in transfected cells and transgenic mice over expressing the human Cu/Zn-superoxide dismutase gene.J Physiol. 1990; 84: 53-77
- Oxidants and the central nervous system.Acta Neurol Scand. 1989; 126: 23-33
- Longitudinal study of neuropsychological function in older adults with Down syndrome.in: Nadel L. Epstein C. Down Syndrome and Alzheimer Disease. Wiley Liss, New York1992: 35-50
- Human chromosome aberrations as related to physical and mental dysfunction.J Hered. 1969; 60: 239
- Transgenic mouse brain histopathology resembles early Alzheimer’s disease.Ann Neurol. 1994; 35: 598-607
- Regular trisomy 21 not accompanied by increased Cu-Zn superoxide dismutase (SOD1) activity.Clin Genet. 1988; 33: 11-19
- Boston Naming Test. Lea & Febiger, Philadelphia1983
- Advances in Alzheimer’s disease.FASEB J. 1991; 5: 278-286
- Down’s syndrome.Free Radic Biol Med. 1988; 4: 317-330
- Down’s syndrome, aging, and Alzheimer’s disease.Ann NY Acad Sci. 1982; 396: 15-27
- Some morphometric observations on the brains of patients with Down syndrome.J Neurol Sci. 1990; 99: 153-164
- Oxidative stress hypothesis in Alzheimer’s disease.Free Radic Biol Med. 1997; 23: 134-147
- Superoxide dismutase and the oxygen enhancement of radiation lethality.Arch Biochem Biophys. 1976; 176: 577-581
- Red cell superoxide dismutase, glutathione peroxidase and catalase in Down’s syndrome patients with and without manifestations of Alzheimer’s disease.Am J Med Genet. 1990; 35: 459-467
- Mutations in Cu/Zn superoxide dismutase gene are associated with familial Amyotrophic lateral sclerosis.Nature. 1993; 362: 59-62
- Alzheimer neuropathology in mentally retarded adults.Acta Neuropathol. 1993; 85: 260-266
- Trisomy 21 (Down’s syndrome) glutathione peroxidase, hexose monophosphate shunt and I.Q.Life Sci. 1979; 24: 29-34
- Down syndrome.Curr Opin Neurol. 1997; 10: 148-152
- Age-related neuropsychological deficits in Down’s syndrome.Biol Psychiatry. 1984; 19: 571-585
- The diagnostic utility of savings score.J Clin Exp Neuropsych. 1993; 15: 773-788
Wechsler D (1981): Wechsler Adult Intelligence Scale-Revised: Manual. New York: Psychological Corporation.
Wechsler D (1987): Wechsler Memory Scale-Revised. New York: Psychological Corporation.
- Detection and staging of dementia in Alzheimer’s disease.Arch Neurol. 1992; 49: 448-452
- Discrepancy between Alzheimer-type neuropathology and dementia in persons with Down’s syndrome.Ann NY Acad Sci. 1986; 477: 247-259
Accepted: January 22, 1999
Received in revised form: January 18, 1999
Received: April 27, 1998
© 1999 Society of Biological Psychiatry. Published by Elsevier Inc. All rights reserved.