Iodotyrosine deiodinase

Iodotyrosine deiodinase is an enzyme that plays a crucial role in the thyroid gland's production of thyroid hormones. This enzyme is responsible for the deiodination of iodotyrosines, which are produced during the synthesis of thyroid hormones.

Function

Iodotyrosine deiodinase is involved in the final step of thyroid hormone synthesis, which is the recycling of iodide. The enzyme catalyzes the deiodination of monoiodotyrosine (MIT) and diiodotyrosine (DIT), which are iodinated tyrosine residues. This process releases iodide, which can then be reused in the synthesis of new thyroid hormones.

Structure

The structure of iodotyrosine deiodinase is complex, with multiple domains that contribute to its function. The enzyme is a member of the NADH oxidase/flavin reductase family of proteins, and it contains a flavin adenine dinucleotide (FAD) binding domain and a substrate-binding domain.

Clinical significance

Mutations in the gene that encodes iodotyrosine deiodinase can lead to a rare disorder known as iodotyrosine deiodinase deficiency. This condition is characterized by hypothyroidism, goiter, and increased levels of iodotyrosine in the urine.

See also

• Thyroid hormone synthesis
• Iodotyrosine
• Hypothyroidism
• Goiter