Gene Symbol: Dio1
Description: deiodinase, iodothyronine, type I
Alias: 5DI, ITDI1, TXDI1, type I iodothyronine deiodinase, DIOI, type 1 DI, type-I 5'-deiodinase
Species: mouse
Products:     Dio1

Top Publications

  1. Hernandez A, Martinez M, Fiering S, Galton V, St Germain D. Type 3 deiodinase is critical for the maturation and function of the thyroid axis. J Clin Invest. 2006;116:476-84 pubmed
    ..These mice provide a new model of central hypothyroidism and reveal a critical role for D3 in the maturation and function of the thyroid axis. ..
  2. Berry M, Grieco D, Taylor B, Maia A, Kieffer J, Beamer W, et al. Physiological and genetic analyses of inbred mouse strains with a type I iodothyronine 5' deiodinase deficiency. J Clin Invest. 1993;92:1517-28 pubmed
    ..We propose the symbol Dio1 to denote the mouse 5'DI gene...
  3. Fonseca T, Fernandes G, McAninch E, Bocco B, Abdalla S, Ribeiro M, et al. Perinatal deiodinase 2 expression in hepatocytes defines epigenetic susceptibility to liver steatosis and obesity. Proc Natl Acad Sci U S A. 2015;112:14018-23 pubmed publisher
    ..The resulting phenotype of the adult ALB-D2KO mouse is dramatic, with greatly reduced susceptibility to diet-induced steatosis, hypertriglyceridemia, and obesity. ..
  4. Toyoda N, Kleinhaus N, Larsen P. The structure of the coding and 5'-flanking region of the type 1 iodothyronine deiodinase (dio1) gene is normal in a patient with suspected congenital dio1 deficiency. J Clin Endocrinol Metab. 1996;81:2121-4 pubmed
    We analyzed the exon-intron structure of the human type 1 deiodinase gene (dio1) and compared it with that of a patient with suspected congenital type 1 deiodinase (D1) deficiency...
  5. Galton V, de Waard E, Parlow A, St Germain D, Hernandez A. Life without the iodothyronine deiodinases. Endocrinology. 2014;155:4081-7 pubmed publisher
  6. Zavacki A, Ying H, Christoffolete M, Aerts G, So E, Harney J, et al. Type 1 iodothyronine deiodinase is a sensitive marker of peripheral thyroid status in the mouse. Endocrinology. 2005;146:1568-75 pubmed
  7. Liao X, Di Cosmo C, Dumitrescu A, Hernandez A, van Sande J, St Germain D, et al. Distinct roles of deiodinases on the phenotype of Mct8 defect: a comparison of eight different mouse genotypes. Endocrinology. 2011;152:1180-91 pubmed publisher
  8. Ohguchi H, Tanaka T, Uchida A, Magoori K, Kudo H, Kim I, et al. Hepatocyte nuclear factor 4alpha contributes to thyroid hormone homeostasis by cooperatively regulating the type 1 iodothyronine deiodinase gene with GATA4 and Kruppel-like transcription factor 9. Mol Cell Biol. 2008;28:3917-31 pubmed publisher
    Type 1 iodothyronine deiodinase (Dio1), a selenoenzyme catalyzing the bioactivation of thyroid hormone, is highly expressed in the liver...
  9. Liu J, Hernandez Ono A, Graham M, Galton V, Ginsberg H. Type 1 Deiodinase Regulates ApoA-I Gene Expression and ApoA-I Synthesis Independent of Thyroid Hormone Signaling. Arterioscler Thromb Vasc Biol. 2016;36:1356-66 pubmed publisher
    ..using liver-specific insulin receptor (InsR) knockout mice identified reduced expression of type 1 deiodinase (Dio1) as a potentially novel link between defective hepatic insulin signaling and reduced expression of the ApoA-I gene...

More Information


  1. Galton V, Hernandez A, St Germain D. The 5'-deiodinases are not essential for the fasting-induced decrease in circulating thyroid hormone levels in male mice: possible roles for the type 3 deiodinase and tissue sequestration of hormone. Endocrinology. 2014;155:3172-81 pubmed publisher
    ..Thus, the systemic changes in thyroid hormone economy as a result of acute food deprivation are not dependent on the D1 or D2 but are mediated in part by sequestration of T4 and T3 in tissues and their enhanced metabolism by the D3. ..
  2. Di Cosmo C, Liao X, Ye H, Ferrara A, Weiss R, Refetoff S, et al. Mct8-deficient mice have increased energy expenditure and reduced fat mass that is abrogated by normalization of serum T3 levels. Endocrinology. 2013;154:4885-95 pubmed publisher
    ..Normalizing serum T3 level by deleting deiodinase 1 corrects body composition and the metabolic alterations caused by the MCT8 deficiency. ..
  3. Charalambous M, Ferron S, da Rocha S, Murray A, Rowland T, Ito M, et al. Imprinted gene dosage is critical for the transition to independent life. Cell Metab. 2012;15:209-21 pubmed publisher
    ..This shows that appropriate dosage control at imprinted loci can act as a critical determinant in postnatal survival during phases of physiological adaptation. ..
  4. Bassett J, Boyde A, Howell P, Bassett R, Galliford T, Archanco M, et al. Optimal bone strength and mineralization requires the type 2 iodothyronine deiodinase in osteoblasts. Proc Natl Acad Sci U S A. 2010;107:7604-9 pubmed publisher
    ..These data reveal an essential role for D2 in osteoblasts in the optimization of bone strength and mineralization. ..
  5. Sund N, Ang S, Sackett S, Shen W, Daigle N, Magnuson M, et al. Hepatocyte nuclear factor 3beta (Foxa2) is dispensable for maintaining the differentiated state of the adult hepatocyte. Mol Cell Biol. 2000;20:5175-83 pubmed
    ..This is the first example of such functional dichotomy for a tissue specification transcription factor. ..
  6. Friedrichsen S, Christ S, Heuer H, Schafer M, Mansouri A, Bauer K, et al. Regulation of iodothyronine deiodinases in the Pax8-/- mouse model of congenital hypothyroidism. Endocrinology. 2003;144:777-84 pubmed
    ..In conclusion, these findings show dramatic age and thyroid state-dependent changes in the expression of deiodinases in central and peripheral tissues of mice during the first 3 wk of life. ..
  7. Klaren P, Looijmans P. Hepatic iodothyronine deiodinase type 1 activity is decreased in two DeltaF508 cystic fibrosis mouse models. J Cyst Fibros. 2004;3:125-8 pubmed
    ..A decreased hepatic D1 activity could be the biochemical basis of some of the abnormal thyroid parameters observed in cystic fibrosis patients. ..
  8. Kelly V, Suzuki T, Nakajima O, Arai T, Tamai Y, Takahashi S, et al. The distal sequence element of the selenocysteine tRNA gene is a tissue-dependent enhancer essential for mouse embryogenesis. Mol Cell Biol. 2005;25:3658-69 pubmed
    ..Together, our data demonstrate that the DSE is a tissue-dependent regulatory element of tRNA(Sec) expression and that its activity is vital for sufficient tRNA(Sec) production during mouse embryogenesis. ..
  9. Maia A, Kieffer J, Harney J, Larsen P. Effect of 3,5,3'-Triiodothyronine (T3) administration on dio1 gene expression and T3 metabolism in normal and type 1 deiodinase-deficient mice. Endocrinology. 1995;136:4842-9 pubmed
    ..In the C3H mouse, hepatic D1 and the dio1 messenger RNA (mRNA) are only 10% that in the C57 strain, the common phenotype...
  10. Selmi Ruby S, Bouazza L, Obregon M, Conscience A, Flamant F, Samarut J, et al. The targeted inactivation of TR? gene in thyroid follicular cells suggests a new mechanism of regulation of thyroid hormone production. Endocrinology. 2014;155:635-46 pubmed publisher
    ..In conclusion, these data show that the control of thyroid hormone production involves not only TR?-dependent mechanisms acting at the level of hypothalamus and pituitary but also TR?-dependent mechanisms acting at the thyroid level. ..
  11. Amendola E, De Luca P, Macchia P, Terracciano D, Rosica A, Chiappetta G, et al. A mouse model demonstrates a multigenic origin of congenital hypothyroidism. Endocrinology. 2005;146:5038-47 pubmed
    ..These results show that TD can be of multigenic origin in mice and strongly suggest that a similar pathogenic mechanism may be observed in humans. ..
  12. Wallis K, Dudazy S, van Hogerlinden M, Nordstrom K, Mittag J, Vennstrom B. The thyroid hormone receptor alpha1 protein is expressed in embryonic postmitotic neurons and persists in most adult neurons. Mol Endocrinol. 2010;24:1904-16 pubmed publisher
    ..Because the role of TR?1 in most neuronal cell types in vivo is largely unknown, our findings suggest that novel functions for thyroid hormone remain to be identified in the brain. ..
  13. Schneider M, Fiering S, Thai B, Wu S, St Germain E, Parlow A, et al. Targeted disruption of the type 1 selenodeiodinase gene (Dio1) results in marked changes in thyroid hormone economy in mice. Endocrinology. 2006;147:580-9 pubmed
    ..the role of the D1 in thyroid hormone economy, a D1-deficient mouse (D1KO) was made by targeted disruption of the Dio1 gene. The general health and reproductive capacity of the D1KO mouse were seemingly unimpaired...
  14. Barca Mayo O, Liao X, Dicosmo C, Dumitrescu A, Moreno Vinasco L, Wade M, et al. Role of type 2 deiodinase in response to acute lung injury (ALI) in mice. Proc Natl Acad Sci U S A. 2011;108:E1321-9 pubmed publisher
    ..We conclude that TH metabolism in the lung is linked to the response to inflammatory injury and speculate that D2 exerts its protective effect by making more TH available to the injured lung tissue. ..
  15. Macek Jílková Z, Pavelka S, Flachs P, Hensler M, Kus V, Kopecky J. Modulation of type I iodothyronine 5'-deiodinase activity in white adipose tissue by nutrition: possible involvement of leptin. Physiol Res. 2010;59:561-9 pubmed
    ..These results suggest a functional role for D1 in white adipose tissue, with D1 possibly being involved in the control of adipose tissue metabolism and/or accumulation of the tissue. ..
  16. Galton V, Schneider M, Clark A, St Germain D. Life without thyroxine to 3,5,3'-triiodothyronine conversion: studies in mice devoid of the 5'-deiodinases. Endocrinology. 2009;150:2957-63 pubmed publisher
  17. Maia A, Berry M, Sabbag R, Harney J, Larsen P. Structural and functional differences in the dio1 gene in mice with inherited type 1 deiodinase deficiency. Mol Endocrinol. 1995;9:969-80 pubmed
    ..The lower D1 levels are paralleled by a decreased normal-sized dio1 mRNA and hyperthyroxinemia...
  18. Amma L, Campos Barros A, Wang Z, Vennstrom B, Forrest D. Distinct tissue-specific roles for thyroid hormone receptors beta and alpha1 in regulation of type 1 deiodinase expression. Mol Endocrinol. 2001;15:467-75 pubmed
    ..Physiologically, these findings suggest a critical role for TRbeta in regulating the thyroid hormone status through D1-mediated metabolism. ..
  19. Waung J, Bassett J, Williams G. Adult mice lacking the type 2 iodothyronine deiodinase have increased subchondral bone but normal articular cartilage. Thyroid. 2015;25:269-77 pubmed publisher
    ..Although adult D2KO mice have normal articular cartilage and no other features of spontaneous joint damage, they exhibit increased subchondral bone mineral content. ..
  20. Ferrara A, Liao X, Gil Ibáñez P, Marcinkowski T, Bernal J, Weiss R, et al. Changes in thyroid status during perinatal development of MCT8-deficient male mice. Endocrinology. 2013;154:2533-41 pubmed publisher
    ..This report is the first to identify that the ontogenesis of TH abnormalities in Mct8-deficient mice manifests with TH excess in the perinatal period...
  21. Schoenmakers C, Pigmans I, Poland A, Visser T. Impairment of the selenoenzyme type I iodothyronine deiodinase in C3H/He mice. Endocrinology. 1993;132:357-61 pubmed
    b>Type I iodothyronine deiodinase (ID-I) activity is impaired in C3H/He (C3H) mice compared with BALB/c and C57BL/6N (C57) mice...
  22. Yu J, Koenig R. Induction of type 1 iodothyronine deiodinase to prevent the nonthyroidal illness syndrome in mice. Endocrinology. 2006;147:3580-5 pubmed
    ..The data suggest that a cytokine-induced defect in T(3) receptor coactivators is an important component of this animal model of nonthyroidal illness and that the syndrome can be overcome by forced expression of the coactivator. ..