1.21.1.1: iodotyrosine deiodinase
This is an abbreviated version!
For detailed information about iodotyrosine deiodinase, go to the full flat file.
Word Map on EC 1.21.1.1
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1.21.1.1
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thyroid
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iodide
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deiodination
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hypothyroidism
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diiodotyrosine
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thyroglobulin
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dehalogenation
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halotyrosines
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iodothyronine
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nitroreductase
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goiter
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bromo
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chlorotyrosine
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organification
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diagnostics
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synthesis
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medicine
- 1.21.1.1
- thyroid
- iodide
-
deiodination
- hypothyroidism
- diiodotyrosine
- thyroglobulin
-
dehalogenation
-
halotyrosines
-
iodothyronine
- nitroreductase
- goiter
-
bromo
-
chlorotyrosine
-
organification
- diagnostics
- synthesis
- medicine
Reaction
Synonyms
DEHAL1, Dehal1 protein, DEHAL1B, EC 1.22.1.1, iodotyrosine dehalogenase 1, iodotyrosine deiodinase, IYD, lyd, NADH oxidase/flavin reductase, SUP-18, SUP-18 IYD, TDH
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General Information
General Information on EC 1.21.1.1 - iodotyrosine deiodinase
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evolution
domain swaps at each N and C terminus consistent with the nitro-FMN reductase superfamily
malfunction
metabolism
physiological function
additional information
increased sup-18(+) expression in body-wall muscles specifically enhances the behavioral defects of sup-10(n983gf) mutants
malfunction
loss-of-function mutations of the enzyme lead to the iodotyrosine deiodinase deficiency (ITDD), characterized by accumulation of mono- and diiodotyrosines in thyroid gland, plasma, and urine, hypothyroidism, compressive goiter and variable mental retardation, whose diagnostic hallmark is the elevation of iodotyrosines in serum and urine. Patients harboring DEHAL1 defects so far described all belong to consanguineous families, phenotype, overview. Lack of biochemical expression of the disease at the beginning of life
malfunction
thyroid dysfunction can have very serious consequences, including mental retardation
genetic analyses suggest that SUP-10 can function with SUP-18 to activate SUP-9 through a pathway that is independent of the presumptive SUP-9 regulatory subunit UNC-93. The SUP-9 two-pore domain K+ channel is most closely related to human TASK-3. unc-93 encodes a conserved multi-pass transmembrane protein. An evolutionarily conserved serine-cysteine-rich region in the C-terminal cytoplasmic domain of SUP-9 is required for its specific activation by SUP-10 and SUP-18 but not by UNC-93
metabolism
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reductive dehalogenation such as that catalyzed by iodotyrosine deiodinase is highly unusual in aerobic organisms but necessary for iodide salvage from iodotyrosine generated during thyroxine biosynthesis
a FMN moiety that is involved in reduced NADPH-dependent reductive deiodination of 3-iodo-Ltyrosine (MIT) and 3,5-diiodo-L-tyrosine (DIT), which are released along with the thyroid hormones T4 and T3 during thyroglobulin proteolysis. Iodotyrosine deiodinase is involved in iodide salvage by catalyzing deiodination of iodinated by-products of thyroid hormone production. Thyroid hormones play important roles in growth, development, differentiation, and basal metabolic homeostasis, as well as in brain development in human fetus and children
physiological function
iodotyrosine deiodinase utilizes FMN to maintain iodide homeostasis by reductive deiodination of iodotyrosine
physiological function
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reductive dehalogenation such as that catalyzed by iodotyrosine deiodinase is highly unusual in aerobic organisms but necessary for iodide salvage from iodotyrosine generated during thyroxine biosynthesis
physiological function
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the enzyme, catalyzing the reductive dehalogenation, is a critical enzyme in maintaining iodine homeostasis
physiological function
the NADH oxidase/flavin reductase, an orthologue of mammalian iodotyrosine deiodinase (IYD), functions in iodine recycling and is important for the biosynthesis of thyroid hormones that regulate metabolism. The enzyme SUP-18 is a type-I transmembrane protein with an NADH oxidase/flavin reductase domain that resides intracellularly and can function without plasma membrane localization. The enzyme regulates the activity of the muscle two-pore domain potassium SUP-9 channel using NADH as a coenzyme and thus couples the metabolic state of muscle cells to muscle membrane excitability
physiological function
the thyroidal enzyme deiodinates mono- and diiodotyrosines (MIT, DIT) and recycles iodine, a scarce element in the environment, for the efficient synthesis of thyroid hormone, function and proposed components of the iodotyrosine deiodinase system, overview
physiological function
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the enzyme, catalyzing the reductive dehalogenation, is a critical enzyme in maintaining iodine homeostasis
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a synergy between substrate selectivity and catalytic activity is created by the enzyme, active site and cofactor and substrate binding structures, overview
additional information
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a synergy between substrate selectivity and catalytic activity is created by the enzyme, active site and cofactor and substrate binding structures, overview
additional information
the human enzyme harbors a conserved nitroreductase domain
additional information
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the human enzyme harbors a conserved nitroreductase domain