Characterization of Channel-Forming Activity in Muscle Biopsy from a Porin-Deficient Human Patient |
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Authors: | Vito De Pinto Angela Messina Angela Schmid Simonetta Simonetti Franco Carnevale Roland Benz |
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Institution: | (1) Department of Chemical Sciences, Laboratory of Biochemistry and Molecular Biology, Università di Catania, viale A. Doria 6, I-95125 Catania, Italy;(2) Lehrstühl für Biotechnologie, Theodor-Boveri-Institut (Biozen trum), Universität Würzburg, Am Hubland, D-97074 Wüzburg, Germany;(3) Division of Metabolic Disease, Hospital Giovanni XXIII, via Amendola, I-70126 Bari, Italy |
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Abstract: | A bioptic specimen from the muscles of a patient suffering from severe myopathy was inspected for the presence of human porin 31HL. Western blotting suggested that the specimen was free of the most abundant eukaryotic porin 31HL (HVDAC1). The specimen was treated with detergent and the soluble protein fraction was passed through a dry hydroxyapatite column. The passthrough of this column was inspected for channel formation in artificial lipid-bilayer membranes. The channel observed under these conditions had a single-channel conductance of about 2.5 nS in 1 M KCl, was cation selective, and was found to be virtually voltage independent. Experiments with a control specimen from a healthy human being, without any indication for muscle myopathy, revealed the presence of the voltage-dependent porin 31HL in the sample. It is discussed whether the patient's bioptic specimen contained another human porin, which has not been studied to date in its natural environment. |
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Keywords: | Porin deficiency muscle biopsy porin isoforms VDAC lipid-bilayer membrane volt age dependence |
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