Pathogenetic Factors in Experimental Galactose Cataract

Abstract

Kalckar et al.^1,2 have recently succeeded in demonstrating the locus of the metabolic block which occurs in the red cell and liver of individuals suffering from congenital galactosemia. A similar block has been demonstrated in the lenses obtained from a child who died of this disease.³ In all these tissues there is a relative or absolute lack of the enzyme galactose-1-phosphate uridyl transferase with the subsequent accumulation of galactose-1-phosphate (Gal-l-P) in the red cell, liver,^1,2 and in the lens.⁴ This ester apparently accumulates to about ten times the level normally present in these tissues.^1,2,4 Since the metabolism of glucose provides the lens with its main source of energy, it seems likely that an accumulation of Gal-1-P within the lens may be the precipitating factor in the development of both the human and the experimental galactose cataract. There are several possible loci where this ester could