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Science 20 September 1974:
Vol. 185. no. 4156, pp. 1053 - 1054
DOI: 10.1126/science.185.4156.1053
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Articles

Type 2 Hyperprolinemia: Absence of utri1-Pyrroline-5-Carboxylic Acid Dehydrogenase Activity

David L. Valle 1, James M. Phang 1, and Stephen I. Goodman 2

1 Metabolism Branch, National Cancer Institute, Bethesda, Maryland 20014
2 Department of Pediatrics, University of Colorado Medical Center, 4200 East Ninth Avenue, Denver 80220

utri1-Pyrroline-5-carboxylic acid dehydrogenase activity was measured radioisotopically in normal and type 2 hyperprolinemia fibroblasts. The type 2 cells had no detectable activity over a range of reaction conditions whereas normal cells had easily measurable activity. This enzymatic defect accounts for the biochemical abnormalities in type 2 hyperprolinemia.


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