Thyroid Hormone Metabolism Defects in a Mouse Model of SBP2 Deficiency.

ABSTRACT: Selenocysteine insertion sequence binding protein 2 (SBP2) is an essential factor in selenoprotein synthesis. Patients with SBP2 defects have a characteristic thyroid phenotype and additional manifestations such as growth delay, male infertility, impaired motor coordination, and developmental delay. The thyroid phenotype has become pathognomonic for this defect, and putative deficiencies in the iodothyronine deiodinases selenoenzymes have been implicated. To investigate the role of SBP2 and selenoproteins in thyroid physiology and answer questions raised by the human syndrome, we generated a tamoxifen-inducible Sbp2 conditional knockout (iCKO) mouse model. These Sbp2-deficient mice have high serum thyroxine (T4), thyrotropin, and reverse triiodothyronine (T3), similar to the human phenotyp

SUBMITTER: Fu J 

PROVIDER: S-EPMC5711384 | biostudies-literature | 2017 Dec

REPOSITORIES: biostudies-literature