The possibility in Myotonic dystrophy (MyD) that a decreased ATP utilization by the membrane may produce modifications in glycoprotein structure and/or in the supramolecular arrangement of some membrane proteins was investigated in human erythrocytes: a) by determining the membrane sialic acid content and the cellular ATP concentration in eight cases of MyD; b) by evaluating the stability of the membrane glycoprotein structure by in vitro ageing experiments; c) by testing the presence of high molecular weight aggregates of proteins on the membrane; d) by evaluating the physico-chemical properties of the membrane by 1-anilino 8-naftalensulfonate as a fluorescent probe. Our evidence suggests that ATP concentration and membrane sialic acid content are within normal values. Only in two cases did a decreased stability of membrane glycoproteins occur while the supramolecular assembly of membrane proteins could be considered as normal. The fluorescent probe behavior after in vitro aging was indicative of a decreased polarity of its micro-environment.

Erythrocyte spectrofluorometric abnormalities in myotonic dystrophy during "in vitro" aging.

BALDUINI, CARLO;SANDRINI, GIORGIO;
1982-01-01

Abstract

The possibility in Myotonic dystrophy (MyD) that a decreased ATP utilization by the membrane may produce modifications in glycoprotein structure and/or in the supramolecular arrangement of some membrane proteins was investigated in human erythrocytes: a) by determining the membrane sialic acid content and the cellular ATP concentration in eight cases of MyD; b) by evaluating the stability of the membrane glycoprotein structure by in vitro ageing experiments; c) by testing the presence of high molecular weight aggregates of proteins on the membrane; d) by evaluating the physico-chemical properties of the membrane by 1-anilino 8-naftalensulfonate as a fluorescent probe. Our evidence suggests that ATP concentration and membrane sialic acid content are within normal values. Only in two cases did a decreased stability of membrane glycoproteins occur while the supramolecular assembly of membrane proteins could be considered as normal. The fluorescent probe behavior after in vitro aging was indicative of a decreased polarity of its micro-environment.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11571/469118
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