A unique electrophoretic slow-moving glucose 6-phosphate dehydrogenase variant (G6PD Asahikawa) with a markedly acidic pH optimum

Takizawa, Takenori; Fujii, H.; Takegawa, Susumu; Takahashi, Kenji; Hirono, Akira; Morisaki, Takayuki; Kanno, Hitoshi; Oka, Ryuichi; Yoshioka, Hidekatsu; Shiro, Motoo

doi:10.1007/bf00293876

Cited by 6 publications

References 13 publications

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Three new G6PD variants, G6PD Adana, G6PD Samanda?, and G6PD Balcali in �ukurova, Turkey

et al. 1987

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Glucose-6-phosphate dehydrogenase (G6PD) enzyme from cases known to be completely or mildly deficient were analyzed. The enzymes were purified from blood samples by utilizing DEAE-52 cellulose pH 7.0 column chromatography and ammonium sulphate precipitation. Biochemical and electrophoretic properties of G6PD were studied in these partially purified enzymes. In this study we report three new variants from Cukurova, named Adana, Samandağ, and Balcali. Variant I (G6PD Adana) had a high Km for G6P (210 microM) and NADP (13 microM). Utilization of 2d-G6P was 38%. It had a slow electrophoretic mobility, a biphasic pH optimum curve, and abnormal heat stability. Variant II (G6PD Samandağ) had a low Km for G6P (25 microM) and a high Km for NADP (18 microM). The rate of utilization of 2d-G6P was normal. G6PD Samandağ deviated from the normal enzyme by its biphasic pH optimum curve and its slow electrophoretic mobility. Variant III (G6PD-Balcali) had a normal Km G6P, NADP and rate of utilization of 2d-G6P. However, it showed a biphasic pH optimum curve and slow electrophoretic mobility.

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Three new G6PD variants, G6PD Adana, G6PD Samanda?, and G6PD Balcali in �ukurova, Turkey

et al. 1987

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Chronic nonspherocytic hemolytic anemia (CNSHA) and glucose 6 phosphate dehydrogenase (G6PD) deficiency in a patient with familial amyloidotic polyneuropathy (FAP)

et al. 1989

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A new glucose-6-phosphate dehydrogenase (G6PD) variant with severe erythrocytic G6PD deficiency and a unique pH optimum is described in a young patient with chronic nonspherocytic hemolytic anemia (CNSHA) and familial amyloidotic polyneuropathy (FAP). Chronic hemolysis was present in the absence of infections, oxidant drugs or ingestion of faba beans. Residual enzyme activity was about 2.6% and 63% of normal activity in erythrocytes and leucocytes, respectively. A molecular study using standard methods showed G6PD in the patient to have normal electrophoretic mobility (at pH 7.0, 8.0 and 8.8), normal apparent affinity for substrates (Km, G6P and NADP) and a slightly abnormal utilization of substrate analogues (decreased deamino-NADP and increased 2-deoxyglucose-6-phosphate utilization). Heat stability was found to be markedly decreased (8% of residual activity after 20 min of incubation at 46 degrees C) and a particular characteristic of this enzyme was a biphasic pH curve with a greatly increased activity at low pH. Although molecular characteristics of this variant closely resemble those of G6PD Bangkok and G6PD Duarte, it can be distinguished from these and all other previously reported variants by virtue of its unusual pH curve. Therefore the present variant has been designated G6PD Clinic to distinguish it from other G6PD variants previously described.

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GLUCOSE-6-PHOSPHATE DEHYDROGENASE VARIANTS IN JAPAN This study was supported in part by research grants from the Ministry of Education, Science and Culture and Ministry of Health and Welfare, Japan.

Shiro

Fujii

1986

Glucose-6-Phosphate Dehydrogenase

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A unique electrophoretic slow-moving glucose 6-phosphate dehydrogenase variant (G6PD Asahikawa) with a markedly acidic pH optimum

Cited by 6 publications

References 13 publications

Three new G6PD variants, G6PD Adana, G6PD Samanda?, and G6PD Balcali in �ukurova, Turkey

Three new G6PD variants, G6PD Adana, G6PD Samanda?, and G6PD Balcali in �ukurova, Turkey

Chronic nonspherocytic hemolytic anemia (CNSHA) and glucose 6 phosphate dehydrogenase (G6PD) deficiency in a patient with familial amyloidotic polyneuropathy (FAP)

GLUCOSE-6-PHOSPHATE DEHYDROGENASE VARIANTS IN JAPAN This study was supported in part by research grants from the Ministry of Education, Science and Culture and Ministry of Health and Welfare, Japan.

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