[1]孙毅娜,叶艳,李永梅,等.甲状腺激素转运体MCT8的病理生理作用[J].国际内分泌代谢杂志,2016,36(01):61-65.[doi:10.3760/cma.j.issn.1673-4157.2016.01.016]
　Sun Yina,Ye Yan,Li Yongmei,et al.Pathophysiological role of monocarboxylate transporter 8 on thyroid hormone transport[J].International Journal of Endocrinology and Metabolism,2016,36(01):61-65.[doi:10.3760/cma.j.issn.1673-4157.2016.01.016]
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甲状腺激素转运体MCT8的病理生理作用()
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《国际内分泌代谢杂志》[ISSN:1673-4157/CN:12-1383/R]

卷:
36
期数:
2016年01期
页码:
61-65
栏目:
综述
出版日期:
2016-01-20

文章信息/Info

Title:
Pathophysiological role of monocarboxylate transporter 8 on thyroid hormone transport
作者:
孙毅娜; 叶艳; 李永梅; 林来祥
300070 天津医科大学代谢病医院内分泌研究所,卫生部激素与发育重点实验室
Author(s):
Sun Yina;  Ye Yan;  Li Yongmei;  Lin Laixiang.
Key Laboratory of Hormones and Development(Ministry of Health), Metabolic Diseases Hospital & Tianjin Institute of Endocrinology, Tianjin Medical University, Tianjin 300070, China
关键词:
甲状腺激素转运体;  艾伦-赫恩登-达得利综合征;  单羧酸转运体8;  有机阴离子转运多肽1C1
Keywords:
Thyroid hormone transporters;  Allan-Herndon-Dudley syndrome;  Monocarboxylate transporter 8;  Organic anion transporting polypeptide 1C1
DOI:
10.3760/cma.j.issn.1673-4157.2016.01.016
摘要:
甲状腺激素(THs)进出细胞需要转运体蛋白的介导。单羧酸转运体(MCT)8是介导T3进入神经元的主要转运体蛋白,是迄今为止唯一具有明确的临床意义、在转运THs入脑中起着重要作用的转运体蛋白,其编码基因(SLC16A2)突变导致了艾伦-赫恩登-达得利综合征(AHDS),以严重的神经运动发育迟滞和高T3、低T4的血清学改变为临床特征。Mct8基因敲除的小鼠模型能够完全复制人MCT8基因突变的血清学改变,但神经症状轻微,部分解释了MCT8缺陷患者的临床表现,为THs转运体病理生理作用的研究提供依据。
Abstract:
Transmembrane protein transporters mediate cellular uptake and efflux of thyroid hormones. Monocarboxylate transporter 8(MCT8)plays an essential role in the supply of T3 to neurons in the central nervous system. So far, MCT8 is the only one with specific clinical significance and can importantlly transport THs into brain. MCT8(encoded gene is SLC16A2)mutations lead to Allan-Herndon-Dudley Syndrome(AHDS)with severe neurological impairment and altered concentrations of thyroid hormones. The endocrine component in Mct8-deficiency mice is likely to be similar to the humans'. However, unlike in humans with an MCT8 deficiency, there is almost not neurological impairment in these mice. After all, deep insight for clinical features with MCT8 mutations can be partly explained and the pathophysiological role of thyroid hormone transporters can be partially identified in Mct8-deficiency mice.

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备注/Memo

备注/Memo:
基金项目:国家自然科学基金资助项目(30901460)
更新日期/Last Update: 2016-01-20