PMID: 9771671Oct 15, 1998

Properties of Ca2+-activated K+ channels in erythrocytes from patients with myotonic muscular dystrophy

Muscle & Nerve
M PellegrinoA Scimemi


Using the single-channel patch-clamp technique, Ca2+-activated K+ channels of erythrocytes from patients with myotonic muscular dystrophy (MyD) were studied. Elementary single-channel properties--conductance, rectification, kinetics, voltage- and calcium-dependence--measured in inside-out patches of MyD erythrocytes, did not differ significantly from those of control cells. The activity of the channels, studied in patches attached to red cells from MyD patients, exhibited mean patch currents which were significantly higher than the controls. The increased mean patch current was due to a higher opening frequency, associated with a reduced mean channel closed time. These results indicate that Ca2+-activated K+ channels of erythrocytes from patients either detect a higher intracellular calcium concentration and/or express an augmented calcium-sensitivity. Since these channels are targets for phosphorylation, our findings make it possible to identify defective kinase mechanisms, in minimally disturbed cells of the patient, at a molecular level of resolution.


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Related Concepts

Calcium [EPC]
Congenital Myotonic Dystrophy
Resting Potentials
Protein Phosphorylation
PDZK1 gene
Clamping Activity
Voltage-Clamp Techniques
Myotonic Dystrophy

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