Abstract
Pendred’s syndrome is characterized by goiter, sensorineural deafness and impaired iodide organification. It is one of the most frequent causes of congenital deafness accounting for about 10% of hereditary hearing loss. It is caused by mutations in the pendrin (PDS) gene, which was postulated to be a sulfate transporter, because of its homology with other genes. We tested sulfate transport in mammalian COS-7 cells that were transiently transfected with PDS cDNA. 35SO4 uptake increased in a time-dependent manner, but this phenomenon was similar in cells transfected with PDS and in mock-transfected cells (450 and 360 cpm/ß-gal units at 10 min, respectively; 38,250 and 31,000 cpm/ß-gal units, at 12 h, respectively). There was no significant increase in 35SO4 uptake using increasing amounts of PDS-containing plasmid (up to 12 μg per dish). These data indicate that pendrin is not a sulfate transporter. Additional functional studies on this protein are warranted to clarify its role in thyroid pathophysiology and inner ear development.
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Bogazzi, F., Bartalena, L., Raggi, F. et al. Pendrin does not increase sulfate uptake in mammalian COS-7 cells. J Endocrinol Invest 23, 170–172 (2000). https://doi.org/10.1007/BF03343701
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DOI: https://doi.org/10.1007/BF03343701