Erythrocyte-ghost Ca2+-stimulated Mg2+-dependent Adenosine Triphosphatase in Duchenne Muscular Dystrophy

Overview

Journal Biochem J

Specialty Biochemistry

Date 1982 Mar 1

PMID 6124239

Citations 2

Authors

M J Dunn

A H Burghes

V Dubowitz

Affiliations

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Abstract

The Ca2+-stimulated Mg2-dependent ATPase activities (Ca2+-ATPase) of erythrocyte-ghost membranes from patients with Duchenne muscular dystrophy (DMD) and carriers of DMD were compared with activities of normal controls. The Ca2+-ATPase activity of DMD-patient ghost preparations was found to follow the same pattern of activation by Ca2+ as the control membranes. However, the Ca2+-ATPase activity in DMD and some DMD-carrier preparations was substantially elevated compared with controls. To characterize further the elevated Ca2+-ATPase activity found in DMD-patient ghost membrane preparations, we estimated kinetic parameters using both fine adjustment and weighting methods to analyse our experimental data. It was established that in both DMD and DMD-carrier preparations the increase in Ca2+-ATPase activity was reflected by a significant increase in Vmax. rather than by any change in Km. The response of the membrane Ca2+-ATPase activity to changes in temperature was also investigated. In all preparations a break in the Arrhenius plot occurred at 20 degrees C, and in DMD and DMD-carrier preparations an elevated Ca2+-ATPase activity was detected at all temperatures. Above 20 degrees C the activation energy for all types of preparation was the same, whereas below this temperature there appeared to be an elevated activation in DMD and DMD-carrier preparations compared with normal controls. The concept that a generalized alteration in the physicochemical nature of the membrane lipid domain may be responsible for the many abnormal membrane properties reported in DMD is discussed.

Citing Articles

Canine malignant hyperthermia susceptibility: erythrocytic defects--osmotic fragility, glucose-6-phosphate dehydrogenase deficiency and abnormal Ca2+ homeostasis.

OBrien P, Forsyth G, Olexson D, Thatte H, Addis P Can J Comp Med. 1984; 48(4):381-9.

PMID: 6150753 PMC: 1236089.

Dystrophin and the integrity of the sarcolemma in Duchenne muscular dystrophy.

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PMID: 2465914 DOI: 10.1007/BF01954866.

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