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. Author manuscript; available in PMC: 2014 Jun 18.
Published in final edited form as: Curr Opin Endocrinol Diabetes Obes. 2013 Oct;20(5):434–440. doi: 10.1097/01.med.0000432531.03233.ad

Table 2.

Characteristics of patients and mice deficient in thyroid hormone cell-membrane transporters

TFTs

FT3 FT4 rT3 TSH HPT CNS Liver Kidney Neurological manifestations Ref.
MCT8-deficient patients ↑↑ ↓↓ nl, sl↑ Resistance Delayed myelination, mild cerebellar atrophy Severe psychomotor impairment (no speech, no walk, poor head control) [24,9]
Mct8 KO mice ↑↑ ↓↓ nl, sl↑ Resistance ↓ T4, ↓ T3, ↑ D2, ↓ D3; ↓RC3 (Striatalneurons) ↑ D1; ↑Gsta2, ↑Gpd2 ↑ T4, ↑ T3, ↑ D1 nl [7,8,31]
Lat2 KO mice nl nl nl nl nl nl D1 nl D1, ↑ Loss of small neutral amino acid Slight impairment of motor coordination on rotarod (caused by aminoacid abnormalities) [15]
Oatp1C1 KO mice nl nl nl ↓ T4, ↓ T3, ↑ D2, ↓ D3 nl D1 nl D1 nl [16▪]
Mct8/Oatp1C1 DKO mice ↓↓ T4, ↓↓ T3, ↑↑ D2; Impaired myelination and neuronal differentiation Coordination and locomotor deficit [42▪▪]

↓, decreased; ↑, increased; CNS, central nervous system; D1, Type 1 deiodinase; D2, Type 2 deiodinase; D3, Type 3 deiodinase; DKO, double knockout; Gpd2, a-glycerol-3-phosphate dehydrogenase; Gsta2, glutathione S transferasea; HPT, hypothalamic–pituitary–thyroid axis; KO, knockout; nl, normal; sl, slight; TFTs, thyroid function tests.