A Coherent Organization of Differentiation Proteins Is Required to Maintain an Appropriate Thyroid Function in the Pendred Thyroid

“…A goiter from a patient with Pendred syndrome has been investigated [10]. First, iodide uptake in methimazole-blocked thyroid slices was similar in the goiter tissue and in normal controls, which does not suggest that one iodide compartment (the follicular lumen) is missing in the Pendred thyroid unless TSH compensation had increased NIS expression and thereby the cellular content.…”

Section: Lessons From the Pendred Syndrome Patientsmentioning

confidence: 99%

“…The pathology of a goiter from a patient with Pendred syndrome gives another view of the problem [10]. In a congenital defect, all the defective cells behave similarly.…”

Section: Lessons From the Pendred Syndrome Patientsmentioning

confidence: 99%

Pendrin: the Thyrocyte Apical Membrane Iodide Transporter?

Twyffels¹,

Massart

Golstein³

et al. 2011

Cell Physiol Biochem

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In the thyroid, the transport of iodide from the extracellular space to the follicular lumen requires two steps: the transport in the cell at the basal side and in the lumen at the apical side. The first step is mediated by the Na⁺/I^- symporter (NIS). In most reviews and textbooks, the second step is presented as mediated by pendrin. In this review, we analyze this assumption. There are several arguments supporting the concept that indeed pendrin plays an important role in thyroid physiology. However, biochemical, clinical and histological data on the thyroid of a patient with Pendred syndrome do not suggest an essential role in iodide transport, which is corroborated by the lack of a thyroid phenotype in pendrin knockout mice. Experiments in vivo and in vitro on polarized and unpolarized cells show that iodide is transported transport of iodide at the apex of the thyroid cell. Moreover, ectopic expression of pendrin in transfected non-thyroid cells is capable of mediating iodide efflux. It is concluded that pendrin may participate in the iodide efflux into thyroid lumen but not as the unique transporter. Moreover, another role of pendrin in mediating Cl^-/HCO₃^- exchange and controlling luminal pH is suggested.

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“…Further functional studies are needed to characterize the potential role of CLCN5 in terms of iodide transport. Interestingly, in thyroid tissue from a patient with Pendred syndrome, the absence of pendrin was accompanied by increased CLCN5 expression [68], and it has been speculated that this may compensate for apical iodide efflux.…”

Section: Controversies Concerning a Physiological Role Of Pendrin In mentioning

confidence: 99%

Controversies Concerning the Role of Pendrin as an Apical Iodide Transporter in Thyroid Follicular Cells

Bizhanova

Kopp

2011

Cell Physiol Biochem

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Pendred syndrome is an autosomal recessive disorder defined by sensorineural deafness, goiter and a partial organification defect of iodide. It is caused by biallelic mutations in the multifunctional anion transporter pendrin/SLC26A4. In human thyroid tissue, pendrin is localized at the apical membrane of thyroid follicular cells. The clinical phenotype of patients with Pendred syndrome and the fact that pendrin can mediate iodide efflux in transfected cells suggest that this anion exchanger may be involved in mediating iodide efflux into the follicular lumen, a key step in thyroid hormone biosynthesis. This concept has, however, been questioned. This review discusses supporting evidence as well as arguments questioning a role of pendrin in mediating iodide efflux in thyrocytes.

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A Coherent Organization of Differentiation Proteins Is Required to Maintain an Appropriate Thyroid Function in the Pendred Thyroid

Cited by 36 publications

References 54 publications

Impact of freezing and thawing of human ovarian tissue on follicular growth after long-term xenotransplantation

Impact of freezing and thawing of human ovarian tissue on follicular growth after long-term xenotransplantation

Pendrin: the Thyrocyte Apical Membrane Iodide Transporter?

Controversies Concerning the Role of Pendrin as an Apical Iodide Transporter in Thyroid Follicular Cells

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