Complete Deficiency of Thyroxine-Binding Globulin (TBG-CD Buffalo) Caused by a New Nonsense Mutation in the Thyroxine-Binding Globulin Gene

Thyroid ◽  
1998 ◽  
Vol 8 (2) ◽  
pp. 161-165 ◽  
Author(s):  
GISAH A. CARVALHO ◽  
ROY E. WEISS ◽  
ADRIAN O. VLADUTIU ◽  
SAMUEL REFETOFF
Keyword(s):  
Nonsense Mutation ◽  
Thyroxine Binding Globulin ◽  
Globulin Gene

Molecular cloning of the rat thyroxine-binding globulin gene and analysis of its promoter activity.

Endocrinology ◽  
1994 ◽  
Vol 135 (6) ◽  
pp. 2731-2736 ◽  
Author(s):  
Y Tani ◽  
Y Mori ◽  
Y Miura ◽  
H Okamoto ◽  
A Inagaki ◽  
...  
Keyword(s):  
Molecular Cloning ◽  
Promoter Activity ◽  
Thyroxine Binding Globulin ◽  
Globulin Gene

scholarly journals Human thyroxine-binding globulin gene: complete sequence and transcriptional regulation.

1993 ◽  
Vol 7 (8) ◽  
pp. 1049-1060 ◽  
Author(s):  
Y Hayashi ◽  
Y Mori ◽  
O E Janssen ◽  
T Sunthornthepvarakul ◽  
R E Weiss ◽  
...  
Keyword(s):  
Transcriptional Regulation ◽  
Complete Sequence ◽  
Thyroxine Binding Globulin ◽  
Globulin Gene

Ataluren: Clinical Trial Results in Nonsense Mutation Duchenne Muscular Dystrophy (nmDMD)

2016 ◽  
Vol 47 (S 01) ◽  
Author(s):  
U. Schara ◽  
C. McDonald ◽  
K. Bushby ◽  
M. Tulinius ◽  
R. Finkel ◽  
...  
Keyword(s):  
Clinical Trial ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Nonsense Mutation ◽  
Clinical Trial Results

Transient Prealbumin- Associated Hyperthyroxinemia in TSH-Producing Pituitary Adenoma

1990 ◽  
Vol 29 (01) ◽  
pp. 40-43 ◽  
Author(s):  
W. Langsteger ◽  
P. Költringer ◽  
P. Wakonig ◽  
B. Eber ◽  
M. Mokry ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Case Report ◽  
Pituitary Adenoma ◽  
Thyroid Hormones ◽  
Protein Binding ◽  
Alpha Subunit ◽  
Normal Range ◽  
Thyroxine Binding Globulin ◽  
Free Thyroid Hormones ◽  
Transmission Computed Tomography

This case report describes a 38-year-old male who was hospitalized for further clarification of clinically mild hyperthyroidism. His increased total hormone levels, the elevated free thyroid hormones and the elevated basal TSH with blunted response to TRH strongly suggested a pituitary adenoma with inappropriate TSH incretion. Transmission computed tomography showed an intrasellar expansion, 16 mm in diameter. The neoplastic TSH production was confirmed by an elevated alpha-subunit and a raised molar alpha-sub/ATSH ratio. However, T4 distribution on prealbumin (PA, TTR), albumin (A) and thyroxine binding globulin (TBG) showed a clearly increased binding to PA (39%), indicating additional prealbumin-associated hyperthyroxinemia. The absolute values of PA, A and TBG were within the normal range. After removal of the TSH-producing adenoma, basal TSH, the free thyroid hormones and T4 binding to prealbumin returned to normal. Therefore, the prealbumin-associated hyperthyroxinemia had to be interpreted as a transitory phenomenon related to secondary hyperthyroidism (T4 shift from thyroxine binding globulin to prealbumin) rather than a genetically conditioned anomaly of protein binding.

THE EFFECT OF ALTERATION OF THYROXINE BINDING CAPACITY ON THE DIALYZABLE AND ABSOLUTE FRACTIONS OF TRIIODOTHYRONINE IN CIRCULATION

1973 ◽  
Vol 72 (2) ◽  
pp. 265-271 ◽  
Author(s):  
J. H. Dussault ◽  
D. A. Fisher ◽  
J. T. Nicoloff ◽  
V. V. Row ◽  
R. Volpe
Keyword(s):  
Correlation Coefficient ◽  
Inverse Relationship ◽  
Binding Capacity ◽  
Hormone Concentration ◽  
Thyroxine Binding Globulin ◽  
Serum Thyroxine ◽  
A Value ◽  
The Mean ◽  
Male Subjects ◽  
Female Subjects

ABSTRACT In order to determine the effect of alterations in binding capacity of thyroxine binding globulin (TBG) on triiodothyronine (T3) metabolism, studies were conducted in 10 patients with idiopathically low (7 subjects) or elevated (3 subjects) TBG levels and 10 subjects given norethandrolone (7 male subjects) or oestrogen (3 female subjects). Measurements of serum thyroxine (T4) concentration, maximal T4 binding capacity, serum T3 concentration and per cent dialyzable T3 were conducted. Serum T3 was measured both by chemical and radioimmunoassay methods. In patients with idiopathically low TBG, the mean serum T4 concentration was low (2.4 μg/100 ml), the mean serum T3 level low (55 ng/100 ml), the mean per cent dialyzable T3 increased (0.52%), and the calculated free T3 concentration normal (186 pg/100 ml). In patients with idiopathically high TBG levels the mean T4 concentration was high (10.3 μg/100 ml), the mean T3 level slightly elevated (127 ng/100 ml), the% dialyzable T3 low (0.10%) and the calculated free T3 concentration low normal (123 pg/100 ml). The correlation coefficient between the per cent dialyzable T3 and maximal TBG binding capacity in the 20 subjects was 0.68, a value significant at the P < 0.01 level. Thus, alterations in binding capacity of TBG seem to influence T3 and T4 metabolism similarly; the inverse relationship between the % of dialyzable hormone and total hormone concentration tends to keep the absolue levels of free hormones stable.

ENDOCRINE CHANGES BEFORE AND AFTER THE MENARCHE

1973 ◽  
Vol 74 (4) ◽  
pp. 685-694 ◽  
Author(s):  
B.-A. Lamberg ◽  
R.-L. Kantero ◽  
P. Saarinen ◽  
O. Widholm
Keyword(s):  
Binding Proteins ◽  
Maturation Process ◽  
Hormone Binding ◽  
Maximal Level ◽  
Continuous Increase ◽  
Mean Values ◽  
Thyroxine Binding Globulin ◽  
Before And After ◽  
Free Thyroxine Index ◽  
Level 1

ABSTRACT In an endocrine survey of healthy girls aged 8 to 20 years before and after the menarche, the serum thyroxine (T4), uptake of triiodothyronine by Sephadex (T3U), and the binding capacities of thyroxine binding globulin (TBG) and pre-albumin (TBPA) were measured, and a free thyroxine index (FTI = T4 × T3U) was calculated. The subjects were grouped according to skeletal age (SA) until the menarche and after this in the post-menarcheal age (PMA), expressed in years. T4 and FTI increased concomitantly and reached peak values of 8.40 μg/100 ml and 8.40, respectively, at 2–3 years PMA. The corresponding mean values for post-menarcheal girls (7.74 μg/100 ml and 7.51) differed statistically significantly from the means before the menarche (7.03 μg/ 100 ml and 6.75). The TBG remained virtually unchanged during the whole period, whereas the TBPA showed a continuous increase and reached a maximal level 1–2 years after the menarche. The maturation process in girls in some way involves an increase in the total and free T4 level which is not dependent on hormone binding proteins.

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