Effects of G-6-PD deficiency, experimentally induced or genetically transmitted, on the sorbitol pathway activity. In vitro and in vivo studies

G Vaca; M G Ramírez; M Vargas; R Mendoza; E Chávez-Anaya; M D Medina; A Alvarez; C Medina; G Sáenz; M Chávez

Effects of G-6-PD deficiency, experimentally induced or genetically transmitted, on the sorbitol pathway activity. In vitro and in vivo studies

Arch Med Res. 1992 Spring;23(1):25-32.

Authors

G Vaca¹, M G Ramírez, M Vargas, R Mendoza, E Chávez-Anaya, M D Medina, A Alvarez, C Medina, G Sáenz, M Chávez

Affiliation

¹ División de Genética, Subjefatura de Investigación Científica, Unidad de Investigación Biomédica, Centro Médico de Occidente, Instituto Mexicano del Seguro Social, Guadalajara, Jalisco.

PMID: 1308788

Abstract

Aldose reductase catalyzes the NADPH-linked reduction of hexoses to their respective sugar-alcohols, which are involved in the pathogenesis of "sugar-cataracts". In the lenses, the reaction catalyzed by G-6-PD is the source of NADPH supply blocking sugar-alcohol formation and consequently prevents or delays the onset of "sugar-cataracts". We have investigated the effect of G-6-PD deficiency, either experimentally induced or genetically transmitted, on the sorbitol accumulation in whole cells incubated in high glucose media and on the "sugar-cataracts" formation in a galactosemic rat model. We also screened 31 Negro male adults with diabetes mellitus for red cell G-6-PD deficiency. G-6-PD deficiency produced a significant inhibition on sorbitol accumulation in rat lenses and human red cells incubated in 50 mM glucose. In the galactosemic rat model G-6-PD deficiency experimentally induced with acetaminophen delayed the development of cataracts. Finally, two diabetic individuals were G-6-PD deficient and did not show cataracts whereas cataracts were identified in six other diabetic patients.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Acetaminophen / pharmacology
Acetaminophen / toxicity
Adolescent
Adult
Aged
Aged, 80 and over
Aldehyde Reductase / metabolism*
Animals
Black People / genetics
Cataract / etiology
Cataract / metabolism
Cataract / prevention & control
Child
Chloramphenicol / pharmacology
Costa Rica / epidemiology
Dehydroepiandrosterone / pharmacology
Diabetes Complications
Diabetes Mellitus / metabolism
Disease Models, Animal
Erythrocytes / metabolism
Galactosemias / complications
Galactosemias / metabolism
Glucosephosphate Dehydrogenase / antagonists & inhibitors
Glucosephosphate Dehydrogenase Deficiency / chemically induced
Glucosephosphate Dehydrogenase Deficiency / complications
Glucosephosphate Dehydrogenase Deficiency / epidemiology
Glucosephosphate Dehydrogenase Deficiency / genetics
Glucosephosphate Dehydrogenase Deficiency / metabolism*
Humans
Incidence
L-Iditol 2-Dehydrogenase / metabolism*
Lens, Crystalline / metabolism
Male
Middle Aged
NADP / metabolism
Oxidation-Reduction
Rats
Rats, Sprague-Dawley
Sorbitol / metabolism*

Substances

Acetaminophen
Dehydroepiandrosterone
Sorbitol
NADP
Chloramphenicol
L-Iditol 2-Dehydrogenase
Aldehyde Reductase
Glucosephosphate Dehydrogenase