Searchable abstracts of presentations at key conferences in endocrinology
Endocrine Abstracts (2014) 35 S3.2 | DOI: 10.1530/endoabs.35.S3.2

ECE2014 Symposia News from thyroid hormones: central transport, energy control and oxidative stress (3 abstracts)

Novel aspects of thyroid hormone transport in the mouse brain

Heike Heuer 1,


1Leibniz Institute for Age Research (FLI), Jena, Germany; 2Leibniz Research Institute for Environmental Medicine (IUF), Düsseldorf, Germany.


Thyroid hormone (TH) actions and metabolism are intracellular events that require the transport of TH across the plasma membrane. This process is facilitated by TH transporters of which the monocarboxylate transporter 8 (MCT8) has been most intensively analyzed. In humans, inactivating mutations in the X-linked MCT8 gene are associated with a severe form of psychomotor retardation in combination with abnormal serum TH parameters. The clinical picture (also known as Allan–Herndon–Dudley syndrome) clearly underscores the significance of MCT8 for proper brain development as well as TH metabolism and function. In mice, however, Mct8 deficiency does not grossly affect brain development whereas the endocrine abnormalities of the patients are fully replicated.

Our studies revealed that in the mouse CNS, another TH transporter is present that can partially compensate for the absence of Mct8. Whereas Mct8 plays a prominent role in facilitating the uptake of the active hormone T3 into the brain, the organic anion transporting peptide Oatp1c1 mediates the transport of T4 across the blood-brain barrier. Consequently, mice deficient in both transporters (Mct8/Oatp1c1 double KO mice) exhibit a pronounced hypothyroid situation in the CNS whereas peripheral organs are in a thyrotoxic state.

Here, I will present a first phenotypic description of Mct8/Oatp1c1 double KO mice that exhibit distinct deficits in neuronal differentiation as well as pronounced locomotor deficiencies. Based on our findings we propose that Mct8/Oatp1c1 double KO mice are a novel animal model for human MCT8 deficiency and a valuable model organism to test possible therapeutic interventions.

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