Red-cell GSH regeneration and glutathione reductase activity in G6PD variants in the Ferrara area.
Red-cell studies were carried out on three groups of G6PD-deficient subjects with different G6PD variants from the Ferrara area of Northern Italy. Red-cell GSH and activities of G6PD, glutathione reductase (GR), glutathione peroxidase (GPx) and superoxide dismutase (SOD) were measured. A method was developed to measure red-cell GSH regeneration after oxidation of endogenous GSH in whole blood by diamide and only this clearly distinguished the variants from each other and from normal. Regeneration by 1 h was lowest in the Mediterranean variant, 0-10.2% in contrast to 93-98% in normal. A predisposition to a haemolytic crisis after ingestion of fava beans was not clearcut, but subjects appeared to be at risk if GSH regeneration at 1 h was less than 30% of the endogenous level, and red-cell FAD+ was very high indicated by high in vitro GR activity and inhibition by added FAD+. It is suggested that the most informative tests in G6PD deficiency are measurements of GSH regeneration in intact red cells plus GR activity and/or red-cell flavin compounds.
Divisione Pediatrica, Arcispedale S. Anna, USL31, Ferrara, Italy.
B B
BB
Anderson
G
G
Carandina
M
M
Lucci
G M
GM
Perry
C
C
Vullo
D000293:Adolescent; D000328:Adult; D004912:Erythrocytes / Q000201:enzymology / Q000378:metabolism*; D005260:Female; D005182:Flavin-Adenine Dinucleotide / Q000097:blood; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000097:blood*; D005978:Glutathione / Q000097:blood*; D005980:Glutathione Reductase / Q000097:blood*; D006801:Humans; D008297:Male; D008875:Middle Aged
D005182:Flavin-Adenine Dinucleotide; D005980:Glutathione Reductase; D005978:Glutathione