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  1. F100 環境医学研究所
  2. F100a 雑誌掲載論文
  3. 学術雑誌

Thyroglobulin gene mutations producing defective intracellular transport of thyroglobulin are associated with increased thyroidal type 2 iodothyronine deiodinase activity

http://hdl.handle.net/2237/8630
7ed126af-de95-4be8-b7a4-86336fe90fa6
名前 / ファイル ライセンス アクション
JCEM-rev-Murata.pdf JCEM-rev-Murata.pdf (3.2 MB)
Item type 学術雑誌論文 / Journal Article(1)
公開日 2007-08-06
タイトル
タイトル Thyroglobulin gene mutations producing defective intracellular transport of thyroglobulin are associated with increased thyroidal type 2 iodothyronine deiodinase activity
その他のタイトル
その他のタイトル High thyroidal D2 activity with Tg mutation (37 characters)
著者 Kanou, Yasuhiko

× Kanou, Yasuhiko

WEKO 18608

Kanou, Yasuhiko

Search repository
Hishinuma, Akira

× Hishinuma, Akira

WEKO 18609

Hishinuma, Akira

Search repository
Tsunekawa, Katsuhiko

× Tsunekawa, Katsuhiko

WEKO 18610

Tsunekawa, Katsuhiko

Search repository
Seki, Koji

× Seki, Koji

WEKO 18611

Seki, Koji

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Mizuno, Yutaka

× Mizuno, Yutaka

WEKO 18612

Mizuno, Yutaka

Search repository
Fujisawa, Haruki

× Fujisawa, Haruki

WEKO 18613

Fujisawa, Haruki

Search repository
Imai, Tsuneo

× Imai, Tsuneo

WEKO 18614

Imai, Tsuneo

Search repository
Miura, Yoshitaka

× Miura, Yoshitaka

WEKO 18615

Miura, Yoshitaka

Search repository
Nagasaka, Tetsuro

× Nagasaka, Tetsuro

WEKO 18616

Nagasaka, Tetsuro

Search repository
Yamada, Chizumi

× Yamada, Chizumi

WEKO 18617

Yamada, Chizumi

Search repository
Ieiri, Tamio

× Ieiri, Tamio

WEKO 18618

Ieiri, Tamio

Search repository
Murakami, Masami

× Murakami, Masami

WEKO 18619

Murakami, Masami

Search repository
Murata, Yoshiharu

× Murata, Yoshiharu

WEKO 18620

Murata, Yoshiharu

Search repository
キーワード
主題Scheme Other
主題 type 2 iodothyronine deiodinase
キーワード
主題Scheme Other
主題 thyroglobulin
キーワード
主題Scheme Other
主題 mutation
キーワード
主題Scheme Other
主題 thyroid gland
キーワード
主題Scheme Other
主題 goiter
抄録
内容記述 Context: Most patients with defective synthesis and/or secretion of thyroglobulin (Tg) present relatively high serum free T3 (FT3) concentrations with disproportionately low free T4 (FT4) resulting in a high FT3/FT4 ratio. The mechanism of this change in FT3/FT4 ratio remains unknown. Objective: We hypothesize that increased type 2 iodothyronine deiodinase (D2) activity in the thyroid gland may explain the higher FT3/FT4 ratio which is frequently observed in patients with abnormal Tg synthesis. Design: We recently identified a compound heterozygous patient (Patient A) with a Tg G2356R mutation and one previously described (C1245R) that is known to cause a defect in intracellular transport of Tg. In the current study, after determining the abnormality caused by G2356R, we measured D2 activity as well as its mRNA level in the thyroid gland. We also measured the thyroidal D2 activity in 3 patients with Tg transport defect and in normal thyroid tissue. Results: Morphological and biochemical analysis of the thyroid gland from Patient A, complemented by a pulse chase experiment revealed that G2356R produces a defect in intracellular Tg transport. D2 activity but not type1 deiodinase in thyroid glands of patients with abnormal Tg transport was significantly higher than in normal thyroid glands, whereas D2 mRNA level in Patient A was comparable with that in normal thyroid glands. Furthermore, there was a positive correlation between D2 activity and FT3/FT4 ratios. Conclusion: Increased thyroidal D2 activity in the thyroid gland is responsible for the higher FT3/FT4 ratios in patients with defective intracellular Tg transport.
内容記述タイプ Abstract
出版者
出版者 The Endocrine Society
言語
言語 eng
資源タイプ
資源タイプresource http://purl.org/coar/resource_type/c_6501
タイプ journal article
DOI
関連識別子
識別子タイプ DOI
関連識別子 http://dx.doi.org/10.1210/jc.2006-1242
ISSN
収録物識別子タイプ ISSN
収録物識別子 0021-972X
書誌情報 Journal of Clinical Endocrinology and Metabolism

巻 92, 号 4, p. 1451-1457, 発行日 2007-04
フォーマット
application/pdf
著者版フラグ
値 author
URI
識別子 http://hdl.handle.net/2237/8630
識別子タイプ HDL
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