Abstract
Thyroid hormone (TH) transporters are required for cellular transmembrane
passage of TH and are thus mandatory for proper TH metabolism and action.
Consequently, inactivating mutations in TH transporters such as MCT8 or
OATP1C1 can cause tissue- specific changes in TH homeostasis. As the most
prominent example, patients with MCT8 mutations exhibit elevated serum T3
levels, whereas their CNS appear to be in a TH deficient state. Here, we
will briefly summarize recent studies of mice lacking Mct8 alone or in
combination with the TH transporters Mct10 or Oatp1c1 that shed light on
many aspects and pathogenic events underlying global MCT8 deficiency and
also underscore the contribution of Mct10 and Oatp1c1 in tissue-specific TH
transport processes. Moreover, development of conditional knock-out mice
that allow a cell-specific inactivation of TH transporters in distinct
tissues, disclosed cell-specific changes in TH signaling, thereby
highlighting the pathophysiological significance of local control of TH
action.
Key words
T3 - T4 - Mct8 - Allan-Herndon-Dudley syndrome - AHDS - Slc16a2 - OATP1C1 - Slco1c1 - MCT10 - Slc16a10