Peroxidase Defect in Congenital Goiter with Complete Organification Block

Nièpomniszcze, Hugo; Castells, Salvador; Groot, Leslie J. De; Refetoff, Samuel; Kim, Ok Soon; Rapoport, Basil; Hati, Ratha N.

doi:10.1210/jcem-36-2-347

Cited by 30 publications

(21 citation statements)

References 0 publications

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“…The ability of the anions used in the test to displace the unbound iodide had been considered to be a mani festation of peroxidase deficiency. Recent biochemical studies have confirmed that iodide peroxidase activity was absent in goitrous cretinism (Haddad and Sidbury 1959;Niepomniszcze et al 1973;Valenta et al 1973) and in patients with goiter, partial perchlorate discharge, euthyroidism and normal hearing (Hagen et al 1971;Niepomniszcze et al 1972). But Pendred's syndrome with goiter, euthyroidism, nerve deafness and partial perchlorate discharge was reported to have a normal peroxidase activity (Burrow et al 1973).…”

Section: In Vivo Studiesmentioning

confidence: 94%

Increased peroxidase activity in Pendred's syndrome with hypothyroidism.

Yamamoto

Saito

Sakurada

et al. 1976

Tohoku J. Exp. Med.

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Section: In Vivo Studiesmentioning

confidence: 94%

Increased peroxidase activity in Pendred's syndrome with hypothyroidism.

Yamamoto

Saito

Sakurada

et al. 1976

Tohoku J. Exp. Med.

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“…Whereas a direct effect of antithyroid drugs can not be ruled out, the peroxidase activity was not changed when rats were sacrificed 1 h after the injection of propylthiouracil (6), although methimazole may behave differently from propylthiouracil (10). However, in the present experi- (11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21). In nontoxic nodular goiter, the activity is decreased in cold nodules and increased or remained the same as normal tissue in warm nodules (11,12).…”

Section: Discussionmentioning

confidence: 56%

“…The localization of peroxidase in thyroid cells is important in relation to its function (26)(27)(28), but only values for total peroxidase activity in thyroid tissue were determined in the present study. It was reported that a small percentage of the total peroxidase activity was found in a fraction sedimenting at 600-1,000 g (5,6,14,15), but the 600-g pellet was discarded in this experiment. Furthermore, iodination of thyroglobulin was studied in vitro without turnover of substrates.…”

Section: Discussionmentioning

confidence: 99%

“…In nontoxic nodular goiter, the activity is decreased in cold nodules and increased or remained the same as normal tissue in warm nodules (11,12). In goiters with an iodide organification defect, peroxidase activity is usually decreased (12)(13)(14)(15)(16)(17), but is normal in Pendred's syndrome (18,19). Nontoxic multinodular goiter has increased peroxidase activity (19,20) and it has been reported in a few patients with toxic diffuse goiter that peroxidase activity per gram tissue is increased much more than DNA content indicating that the activity per cell is increased (12,19,21 The administration of TSH stimulates thyroidal peroxidase activity in rats (6,22,23); however, the increase in peroxidase activity in thyroids of patients with Graves' disease is not due to an increase in serum TSH since TSH, T4, and T3 levels in the patients reported in this study were within the normal ranges and serum TSH concentrations were not detectable after the administration of T3.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Iodinating activity of thyroid tissue in toxic diffuse goiter.

Nagataki¹,

Uchimura²,

Ikeda³

et al. 1977

J. Clin. Invest.

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A B S T R A C T Thyroid tissue obtained from 12 patients with Graves' disease and treated with thionamide drugs for 3-7 mo before subtotal thyroidectomy, from 12 patients with Graves' disease, similarly treated, and given 50 ,ug of triiodothyronine (T3) for 10 days before surgery, and from 12 euthyroid patients with solitary cold nodules was investigated to compare in vitro iodination of thyroglobulin in toxic diffuse goiter and in normal thyroid tissue. The supernates of the homogenates (105,000g) were subjected to sucrose density gradient centrifugation (5-28%) to separate the thyroglobulin fraction. The precipitates were treated with 1% digitonin and centrifuged to collect the supernate (particulate fraction). When thyroglobulin and particulate fractions obtained from the same patient were incubated with 125I-, iodide, glucose, and glucose oxidase, the amount of iodine bound to thyroglobulin was several times greater in toxic diffuse goiter than in normal thyroid tissue; administration of T3 did not affect iodination in toxic diffuse goiter. When the thyroglobulin fraction from each patient was incubated with a standardized quantity of peroxidase instead of the individual particulate fraction, the amount of iodine bound to thyroglobulin was the same among the three groups of patients. Finally, when bovine serum albumin was substituted for thyroglobulin from each of the patients, iodination of bovine serum albumin was several times greater with the particulate fraction obtained from toxic diffuse goiter tissue than with that obtained from normal

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“…Complete absence of thyroid peroxidase activity has been described in patients with TIODs (7)(8)(9).…”

Section: T Hyroid Peroxidase (Tpo) Is a Membrane-linkedmentioning

confidence: 99%