The hexose monophosphate pentose pathway in fibroblasts deficient in L-ornithine: 2-oxoacid aminotransferase activity.

Abstract Δ 1 -pyrroline-5-carboxylate has been shown to exert a strong stimulatory effect on the hexose monophosphate pentose pathway of glucose oxidation in fibroblasts. In gyrate atrophy, activity of an enzyme which can form Δ 1 -pyrroline-5-carboxylate is absent. The effect of this deficiency on the operation of the hexose monophosphate pentose pathway in fibroblasts from gyrate atrophy patients has not been examined. This communication describes such a study and shows that glucose metabolism through this pathway is the same for gyrate atrophy and normal fibroblasts either in the presence or absence of added Δ 1 -pyrroline-5-carboxylate.