Journal article
Amino-acid metabolism and liver ultrastructure in hyperornithinemia with gyrate atrophy of the choroid and retina
Abstract
Three patients with the rare hyperornithinemia with gyrate atrophy of the choroid and retina (HOGA) syndrome were studied to elucidate the metabolic derangement and its pathologic concomitants. Tenfold elevations of blood ornithine levels, decreases in lysine levels, and hitherto unreported decreases in blood glutamate and glutamine concentration were observed. The output of ornithine from muscle kidney and splanchnic beds was curtailed or …
Authors
Arshinoff SA; McCulloch JC; Matuk Y; Phillips MJ; Gordon BA; Marliss EB
Journal
Metabolism, Vol. 28, No. 10, pp. 979–988
Publisher
Elsevier
Publication Date
October 1979
DOI
10.1016/0026-0495(79)90001-5
ISSN
0026-0495