Abstract
1. (1) The activity of glucose-6-phosphatase (G6Pase) was assayed in human, guinea pig and rat jejunum. A specific G6Pase was indicated in human and guinea pig jejunum but not in that of the rat. 2. (2) Two cases of glycogen storage disease of Con's type 1 were shown completely to lack jejunal G6Pase activity. In spite of this enzyme deficiency they did not have a pathological glycogen storage in the jejunum. 3. (3) It is concluded that human jejunal G6Pase may be governed by the same gene as liver and kidney G6Pase. 4. (4) The importance of the enzyme in human jejunal mucosa is discussed, especially with regard to glucose transport and fructose-to-glucose conversion. 5. (5) Peroral jejunal mucosa biopsy in patients suspected to suffer from von Gierke's disease is judged to be a diagnostic possibility, when liver biopsy cannot be performed.
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