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February 1971

Ouabain and Erythrocyte-Ghost Adenosine Triphosphatase: Effects in Human Muscular Dystrophies

Author Affiliations

Fukuoka City, Japan
From the Department of Neurology, Neurological Institute, Faculty of Medicine, Kyushu University, Fukuoka City, Japan.

Arch Neurol. 1971;24(2):187-190. doi:10.1001/archneur.1971.00480320115012

An adenosine triphosphatase (ATPase) of erythrocyte ghosts from all six patients with Duchenne dystrophy and three of six patients with limb-girdle dystrophy was stimulated by 10-4 molar ouabain but inhibited by the same concentration of ouabain in 11 normal subjects, one patient with Becker dystrophy, and eight other neurological or muscular disorders. The inhibited response was also observed in two possible carriers of Duchenne dystrophy. The abnormality of erythrocyte ghost ATPase in patients with Duchenne dystrophy was not related to age, disability, or serum creatine phosphokinase. The mechanism of the abnormality of ATPase in erythrocyte ghosts from all patients with Duchenne dystrophy and some patients, but not all, with limb-girdle dystrophy is not known (24:187-200, 1971).

Key Words.—  Ouabain, erythrocyte-ghost ATPase, membrane ATPase, sodium-potassium-ATPase, human muscular dystrophies, Duchenne dystrophy, limb-girdle dystrophy.

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