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454068 
Journal Article 
Review 
Minireview: The Sodium-Iodide Symporter NIS and Pendrin in Iodide Homeostasis of the Thyroid 
Bizhanova, A; Kopp, P 
2009 
Yes 
Endocrinology
ISSN: 0013-7227
EISSN: 1945-7170 
ENDOCRINE SOC 
WASHINGTON 
150 
3 
1084-1090 
English 
19196800 
WOS:000263613200004 
Thyroid hormones are essential for normal development and metabolism. Thyroid hormone biosynthesis requires iodide uptake into the thyrocytes and efflux into the follicular lumen, where it is organified on selected tyrosyls of thyroglobulin. Uptake of iodide into the thyrocytes is mediated by an intrinsic membrane glycoprotein, the sodium-iodide symporter (NIS), which actively cotransports two sodium cations per each iodide anion. NIS-mediated transport of iodide is driven by the electrochemical sodium gradient generated by the Na+/K+-ATPase. NIS is expressed in the thyroid, the salivary glands, gastric mucosa, and the lactating mammary gland. TSH and iodide regulate iodide accumulation by modulating NIS activity via transcriptional and posttranscriptional mechanisms. Biallelic mutations in the NIS gene lead to a congenital iodide transport defect, an autosomal recessive condition characterized by hypothyroidism, goiter, low thyroid iodide uptake, and a low saliva/plasma iodide ratio. Pendrin is an anion transporter that is predominantly expressed in the inner ear, the thyroid, and the kidney. Biallelic mutations in the SLC26A4 gene lead to Pendred syndrome, an autosomal recessive disorder characterized by sensorineural deafness, goiter, and impaired iodide organification. In thyroid follicular cells, pendrin is expressed at the apical membrane. Functional in vitro data and the impaired iodide organification observed in patients with Pendred syndrome support a role of pendrin as an apical iodide transporter. (Endocrinology 150: 1084-1090, 2009) 
syndrome gene pds; sodium/iodide symporter; na+/i-symporter; molecular; analysis; plasma-membrane; frtl-5 cells; congenital deafness; inner-ear; vestibular aqueduct; anion transporters