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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REFERENCES
Báthori, G., Parolini, I., Tombola, F., Szabó I., Messina, A., Oliva, M., De Pinto, V., Lisanti, M., Sargiácomo, M., and Zoratti, M. (1999) J. Biol. Chem. 274, 29607-29612.
Benz, R. (1994). Biochim. Biophys. Acta 1197, 167-196.
Benz, R., Janko, K., Boos, W., and Laüger, P. (1978). Biochim. Biophys. Acta 511, 305-319.
Benz, R., Janko, K., and Lüuger, P. (1979). Biochim. Biophys. Acta 551, 238-247.
Benz, R., Maier, E., Thinnes, F. P., Goetz, H., and Hilschmann, N. (1992). Biol. Chem. Hoppe-Seyler 373, 295-303.
Blachly-Dyson, E., Zambronicz, E. B., Yu, W. H., Adams, V., McCabe, E. R. B., Adelman, J., Colombini, M., and Forte, M. (1993). J. Biol. Chem. 268, 1835-1841.
Colombini, M. (1979). Nature (London) 279, 643-645.
De Pinto, V., Prezioso, G., and Palmieri, F. (1987). Biochim. Biophys. Acta 905, 499-502.
De Pinto, V., Benz, R., Caggese, C., and Palmieri, F. (1989a). Biochim. Biophys. Acta 987, 1-7.
De Pinto, V., Benz, R., and Palmieri, F. (1989b). Eur. J. Biochem. 183, 179-187.
De Pinto, V., Prezioso, G., Thinnes, F., Link, T. A., and Palmieri, F. (1991). Biochemistry 30, 10191-10200.
Freitag, H., Neupert, W., and Benz, R. (1982). Eur. J. Biochem. 123, 629-636.
Huizing, M., Ruitenbeek, W., Thinnes, F. P., and De Pinto, V. (1994). Lancet 344, 762.
Huizing, M., Ruitenbeek, W., Thinnes, F. P., De Pinto, V., Wendel, U., Trijbels, F. J. M., Smit L. M. E., ter Laak, H. J., and van der Heuvel, L. P. (1996). Pediatric Res. 39, 1-6.
Junankar, P. R., Dulhunty, A. F., Curtis, S. M., Pace, S. M., and Thinnes, F. P. (1995). J. Muscle Res. Cell Motility 16, 595-610.
Lewis, T. M., Roberts, M. L., and Bretag, A. H. (1994). Neurosci. Lett. 181, 83-86.
Lindèn, M., Gellerfors, M., and Nelson, B. D. (1982). Biochem. J. 208, 77-82.
Mannella, C. A., Guo, X. W., and Cognon, B. (1989). FEBS Lett. 253, 231-234.
Messina, A., Oliva, M., Rosato, C., Huizing, M., van der Heuvel, L. P., Forte, M., Rocchi, M., and De Pinto, V. (1998). Biochem. Biophys. Res. Commun. 255, 707-710.
Popp, B., Schmid, A., and Benz, R. (1995). Biochemistry 34, 3352-3361.
Popp, B., Court, D. A., Benz, R., Neupert W., and Lill, R. (1996). J. Biol. Chem. 271, 13593-23599.
Rahmani, J., Maunoury, C., and Siddiqui, A. (1998). Eur. J. Human Genet. 6, 337-340.
Reymann, S., Haase, W., Krick, W., Burchardt, G., and Thinnes, F. P. (1998). Pflügers Arch. Eur. J. Physiol. 436, 478-480.
Roos, N., Benz, R., and Brdiczka, D. (1982). Biochim. Biophys. Acta 686, 204-214.
Sampson, M. J., Lovell, R. S., and Craigen, W. J. (1997). J. Biol. Chem. 272, 18966-18973.
Sampson, M. J., Ross, L. O., Decker, W. K., and Craigen, W. J. (1998). J. Biol. Chem. 273, 30482-30468.
Thinnes, F. P., Goetz, H., Kayser, H., Benz, R., Schmidt, W. E., Kratzin, H., and Hilschmann, N. (1989). Biol. Chem. Hoppe-Seyler 370, 1253-1264.
Xu, X., Decker, W., Sampson, M. J., Craigen, W. J., and Colombini, M. (1999). J. Membr. Biol. 170, 89-102.
Yu, W. H., Wolfgang, W., and Forte, M. (1995). J. Biol. Chem. 270, 13998-14006.
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De Pinto, V., Messina, A., Schmid, A. et al. Characterization of Channel-Forming Activity in Muscle Biopsy from a Porin-Deficient Human Patient. J Bioenerg Biomembr 32, 585–593 (2000). https://doi.org/10.1023/A:1005622611410
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DOI: https://doi.org/10.1023/A:1005622611410