Type I iodothyronine deiodinase

UniProt P49895 Organism Homo sapiens Gene DIO1, ITDI1, TXDI1 EC 1.21.99.3, 1.21.99.4

Also known as: DIO1, ITDI1, TXDI1

Function

Plays a crucial role in the metabolism of thyroid hormones (TH) and has specific roles in TH activation and inactivation by deiodination. Catalyzes the deiodination of L-thyroxine (T4) to 3,5,3'-triiodothyronine (T3), 3,3',5'-triiodothyronine (rT3) to 3,3'-diiodothyronine (3,3'-T2) and 3',5'-diiodothyronine (3',5'-T2) to 3'-monoiodothyronine (3'-T1) via outer-ring deiodination (ORD). Catalyzes the deiodination of T4 to 3,3',5'-triiodothyronine (rT3) via inner-ring deiodination (IRD). Catalyzes the deiodination of T3 to 3,3'-T2, 3,5-diiodothyronine (3,5-T2) to 3- monoiodothyronine (3-T1) and 3,3'-T2 to 3-T1 via IRD (By similarity). Catalyzes the phenolic ring deiodinations of 3,3',5'-triiodothyronamine and 3',5'-diiodothyronamine. Catalyzes the phenolic ring deiodination of 3,3'-diiodothyronamine and tyrosyl ring deiodinations of 3,5,3'-triiodothyronamine and 3,5-diiodothyronamine (By similarity). Catalyzes the deiodination of L-thyroxine sulfate and 3,3',5-triiodo-L-thyronine sulfate via IRD and of 3,3',5'-triiodo-L-thyronine sulfate via ORD (By similarity).

Classification

Family (Pfam): PF00837 T4_deiodinase
InterPro: Iodothyronine_deiodinase, Iodothyronine_deiodinase_AS, Iodothyronine_deiodinase_I/III, Thioredoxin-like_sf
Functional cluster: Zinc-Finger & Ubiquitination Proteins

Gene Ontology · 11

Disease associations

thyroid hormone metabolism, abnormal, 2 MONDO:0030839

Drugs targeting this protein · 1

PROPYLTHIOURACIL inhibitor

Related proteins · sequence + function similarity

Co-cited proteins · studied together in the literature

Type I iodothyronine deiodinase 3 shared papers
Thyroxine 5-deiodinase 3 shared papers
Type II iodothyronine deiodinase 3 shared papers

Literature · 8 cited papers

Human Type 1 Iodothyronine Deiodinase (DIO1) Mutations Cause Abnormal Thyroid Hormone Metabolism. Thyroid · 2021
Development of a validated liquid chromatography/tandem mass spectrometry method for the distinction of thyronine and thyronamine constitutional isomers and for the identification of new deiodinase substrates. Rapid Commun. Mass Spectrom. · 2008
Thyronamines are isozyme-specific substrates of deiodinases. Endocrinology · 2008
The DNA sequence and biological annotation of human chromosome 1. Nature · 2006
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
In vivo dimerization of types 1, 2, and 3 iodothyronine selenodeiodinases. Endocrinology · 2003
A novel retinoid X receptor-independent thyroid hormone response element is present in the human type 1 deiodinase gene. Mol. Cell. Biol. · 1995
Cloning and in vitro expression of the human selenoprotein, type I iodothyronine deiodinase. J. Clin. Endocrinol. Metab. · 1992

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