Goitre and Cretinism Due to the Production of an Abnormal Iodinated Thyroid Compound

McGirr, E. M.; Hutchison, John A.; Clement, W.Elspeth; Kennedy, J. S.; Currie, A. R.

doi:10.1177/003693306000500501

Cited by 26 publications

(9 citation statements)

References 22 publications

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“…This iodoalbumin has ialso been identified from the serum of the patients (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15). From the poorly iodinated iodoalbumin, low peptide-linked levels of hormone were already reported (8,11).…”

Section: Discussionmentioning

confidence: 99%

“…BII compounds, other than the circulating iodoalbumin, were often mentioned in this variety of congenital goiter as unidentified and puzzling substances. They were found after hydrolysis of the serum (1) or the thyroidal iodoproteins (7) as "resistant to digestion" material, remaining at the origin of the chromatograms (2) or insoluble in acid butanol (9). They were detected in the urine of patients (8,11) and presumed to be shortchain polypeptides (2), suggesting a iodoprotein breakdown defect.…”

Section: Discussionmentioning

confidence: 99%

“…The iodotyrosine deiodinase peripheral activity was determined in vivo from labeled MIT or DIT in the conditions described (12). The (15); BII from plasma by a modification of a described method (9).…”

Section: Methodsmentioning

confidence: 99%

“…The circulating iodo-compound was identified, at least in part, as iodoalbumin (3)(4)(5). An iodoalbumin has been identified in similar goiters (6), and thyroglobulin has been reported as reduced (3)(4)(5)7) or apparently absent (2,(8)(9)(10)(11). From similar patients, other butanol-insoluble compounds were detected either in thyroid (2,4,7) or serum (1,4,8) hydrolysates, and in urines (2,3,10,11).…”

Section: Introductionmentioning

confidence: 94%

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Studies on Mono- and Diiodohistidine. II. CONGENITAL GOITROUS HYPOTHYROIDISM WITH THYROGLOBULIN DEFECT AND IODOHISTIDINE-RICH IODOALBUMIN PRODUCTION

Savoie¹,

Massin²,

Savoie³

1973

J. Clin. Invest.

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A B S T R A C T Butanol-insoluble iodinated compounds in the urine of patients with congenital goiters have been generally regarded as iodopeptides. Monoiodohistidine (MIH) and diiodohistidine (DIH) were identified from the urine of four patients with congenital goitrous hypothyroidism. From radioiodine studies, 40-70% of the urinary radioactivity was in the iodide-free fraction from which about 40% was identified as MIH and DIH by crystallizations to a constant specific activity.Iodotyrosines were simultaneously identified in the urine. However the presence of an iodotyrosine-deiodinase activity was demonstrated in the two removed goiters with a normal Km for MIT. In vivo iodotyrosine deiodination was normal for hypothyroid subjects.No thyroglobulin was identified in the thyroids from these patients. The major iodoprotein was iodoalbumin which, after in vivo labeling, contained 84-89% of the total soluble protein radioactivity. The thyroxine content of the goiter iodoalbumins and other iodoproteins was extremely low.Iodohistidines were identified in comparable proportions in the iodoalbumin and in the other iodoproteins isolated from each goiter. The average iodohistidine content of these proteins as crystallizable MIH and DIH was in the individual cases 15 and 4% of the in vivo incorporated radioiodine. DIH was identified in all iodoprotein fractions. The mean DIH/MIH ratios from the individual cases were 1.16 and 0.35. The corresponding DIT/MIT ratios were 3.19 and 1.45, respectively.The major consequence of this thyroglobulin defect is the iodination of inappropriate proteins (mainly albumin) resulting in low yields of thyroxine and high Received for publication 6 March 1972 and in revised form 19 September 1972. yields of iodohistidines. Iodohistidines from the goiter iodoproteins were not deiodinated and, at least for MIH, were quantitatively excreted in the urine of these patients. From the MIH iodoalbumin content and the MIH urinary excretion, goiter iodoalbumin turnover estimates were made and, although elevated, could not maintain a normal thyroxine secretion.The urinary excretion of iodohistidines easily demonstrated by column chromatography is offered as a test for detecting this variety of congenital goiter.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 94%

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Studies on Mono- and Diiodohistidine. II. CONGENITAL GOITROUS HYPOTHYROIDISM WITH THYROGLOBULIN DEFECT AND IODOHISTIDINE-RICH IODOALBUMIN PRODUCTION

Savoie¹,

Massin²,

Savoie³

1973

J. Clin. Invest.

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“…Most of these proteins have been characterized as serum proteins, either diffused from circulation or synthesized by the follicular cells, aspecifically iodinated on account of the strikingly defective TG formation [7,10,[16][17][18]20], However, it has been demonstrated that some of the soluble, slow-sedimenting iodoproteins are immunologically related to TG [2,4,6]. The precursor-product relationship between parti culate iodoproteins and soluble TG has been assumed on the basis of data obtained from normal rats [12] and on an experimental rat thyroid tumor [21,27], but evidence for possible correlation in human thyroid diseases between the particulate iodoproteins and TG secreted in the follicular lumen has not been supported by convincing data [19,26].…”

Section: Introductionmentioning

confidence: 99%

Isolation and Characterization of Soluble and Particulate Thyroid Iodoproteins in Human Congenital Goiter

Monaco¹,

Andreoli²,

A³

1974

Horm Res

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Soluble and particulate iodoproteins solubilized by detergent from in vivo ¹²⁵I labeled congenital goiter, have been isolated and characterized. The soluble iodoproteins (S₁) were decreased but contained 80% of radioiodine; 19S thyroglobulin (TG) represented only 9% of the soluble extract, the main component being iodinated serum albumin. The solubilized iodoproteins (S₂) consisted of TG-like material and represented one third of the total TG, sedimented as 19S and possessed the immunoreactive properties of native TG. The iodine content of TG from S₁ and S₂ was 0.01% and 0.008%, respectively, and no radio-labeled truodothyronine or tetraiodothyronine was detectable in the two TG containing only monoiodotyrosine and diiodotyrosine. S₁ and S₂ TG showed the same resistance to dissociation at alkaline pH in low salt. The sialic acid content was markedly decreased in both S₁ and S₂ TG, the particulate protein contained 0.06%, compared to 0.12% found in the soluble goitrous fraction and to 1.3% described in the normal soluble TG. The results presented, whilst confirming the striking decrease of soluble TG and the increase in the particulate iodoproteins, lend support to the hypothesis that TG synthesis is impaired in some cases of human congenital goiter. Moreover, our findings demonstrate the presence of membrane-bound 19S molecules and a decreased sialic acid content in the goitrous TG, which is more marked in the particulate fraction. These results suggest that a defective secretion into the follicular lumen of fully synthesized TG could be responsible for the dishormonogenetic errors in some cases of congenital goiter.

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Epithelial binding of radio‐iodine in human goitre due to dyshormonogenesis

1965

J. Pathol.

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Goitre and Cretinism Due to the Production of an Abnormal Iodinated Thyroid Compound

Cited by 26 publications

References 22 publications

Studies on Mono- and Diiodohistidine. II. CONGENITAL GOITROUS HYPOTHYROIDISM WITH THYROGLOBULIN DEFECT AND IODOHISTIDINE-RICH IODOALBUMIN PRODUCTION

Studies on Mono- and Diiodohistidine. II. CONGENITAL GOITROUS HYPOTHYROIDISM WITH THYROGLOBULIN DEFECT AND IODOHISTIDINE-RICH IODOALBUMIN PRODUCTION

Isolation and Characterization of Soluble and Particulate Thyroid Iodoproteins in Human Congenital Goiter

Epithelial binding of radio‐iodine in human goitre due to dyshormonogenesis

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