α-Glucosidase deficiency in generalized glycogen-storage disease (Pompe's disease)

Abstract

CATECHOL COMPOUNDS IN BRAINS11 Schiimann (1959) identified the amine in seven organs of dog, sheep and ox, and found that the concentration of 3-hydroxytyramine relative to that of noradrenaline in brains of dogs was ex- ceeded in liver and intestine, whereas Montagu (1957, 1959) and Bertler & Rosengren (1959) found only smaller and 'very small' amounts respectively in tissues of non-ruminant animals other than brain, suggesting a special role in this organ.Unlike noradrenaline and adrenaline (Vogt, 1954), the concentration of hydroxytyramine is highest in the corpus striatum (Bertler & Rosengren, 1959;Ehringer & Hornykiewicz, 1960).3,4-Dihydroxyphenylalanine and the acidic catechol compound have not been identified in other than human brains or in rat tissues, but even quite large amounts, especially of 3,4-dihydroxyphenylalanine, could escape detection in analyses of extracts by the ethylenediamine method.The estimated concentration of the acidic catechol compound was about the same in the first human brain and in the sample from the second, but there was no evidence for the presence of 3,4-dihydroxyphenylalanine in the latter.Since there were (un- identified) non-basic catechol compounds in rabbit brains, which were extracted immediately after death, it seems unlikely that their presence in human brain is due to post-mortem change.SUMMARY 1. 3-Hydroxytyramine constituted between 50 and 80% of the total catecholamine concentration of the whole brains of male rats, guinea pigs, rabbits, cockerels and man.In human brain 3,4- dihydroxyphenylalanine and an acidic catechol compound were also present.I thank Dr H. Weil