Taurine Excretion and Intelligence in Mongolism.

Abstract

Mean urinary taurine excretion in mongolism is significantly less than that of normal controls( 1). This difference is due in part to the fact that a proportion of mongoloids excrete taurine below the lowest level observed among normal subjects. The difference is believed to be genetically controlled and is compatible with an overdose effect ( 2 ) .It was noted that .mongoloids with lowest excretion levels tended to be residents of cottages housing patients who are non-ambulatory, profoundly retarded or both. Cottage placement for individuals without physical disabilities is primarily dependent on level of adaptive behavior which is in part determined by their level of intellectual function. These facts encouraged study of the relationships between taurine excretion and cottage placement and between taurine excretion and I.Q. scores.Methods. The clinical impression of mongolism was confirmed by karyotyping all 41 mongoloid subjects. Accurate 24-hour urines were collected at the infirmary of the Western Carolina Center. Taurine was determined as previously described (3) and creatinine according to Taussky's procedure (4).The results of previous psychological tests were reviewed by a clinical psychologist at Western Carolina Center. Subjects were retested if previous test results were over 2 years old and the subject was less than 16 years old a t time of testing. Tests were also repeated if the previous examiner considered the results invalid or if a range rather than a specific score had been recorded. The Vineland Social Maturity Scale (S.Q.) was used for 11 subjects who were either nm-verbal or had a basal age of less than 2 years according to the Stanford-Binet. The Stanford-Binet (Form LM) was used with the 30 re-*This work was supported in part by U.S.P.H.S.

Prenatal Organ and Cellular Growth with Various Chromosomal Disorders

“…Blood levels of urea nitrogen, uric acid and creatinine are all abnormally increased in trisomy G" another evidence that protein and cellular turnover may be increased [1,3,9,12,15]. A wide variety of other protein and enzymatic abnormalities have also been recorded in mongols in recent years; many suggest disturbed cellular function which might affect either cellular multiplication or survival [25,30,36,37,39],…”

Subcellular distribution of taurine and cysteinesulphinate decarboxylase in developing rat brain

“…Under the control of adrenaline and acetylcholine, isethionic acid is formed from taurine in dog heart, where it has a profound effect on K+ retention (Welty & Read, 1964). If such a mechanism operates in the brain, especially in the young, any defect in taurine metabolism or transport, as may be found in mongolism (Thomas, Goodman, King & Wainer, 1965) may have serious consequences for the functioning of nerve cells. In analogy with experiments with phenylalanine ) L. K. Kaczmarek (unpublished work) has tried to interfere with the normal balance of taurine transport by considerably raising plasma taurine concentrations and have shown that this has no effect on the transport or utilization in protein synthesis of a related amino acid, glycine.…”

Urinary levels of taurine in mentally retarded children

“…Associations significant at the 5% level, were found between the levels of urinary taurine and two measures of adaptive behaviour as an indication of intelligence. Calculations indicated that 10-20% of the reduction in intelligence could be accounted for by the observed correlations (Thomas et al, 1965), with a number of assumptions concerning this extrapolation. Thus, the preliminary fmdings of the present study indicate a highly integrated relationship between the urinary taurine levels and mental retardation.…”