Congenital hypothyroid Pax8-/- mutant mice can be rescued by inactivating the TRα gene

被引：155

作者：

Flamant, F

Poguet, AL

Plateroti, M

Chassande, O

Gauthier, K

Streichenberger, N

Mansouri, A

Samarut, J

机构：

[1] Ecole Normale Super Lyon, Biol Cellulaire & Mol Lab, CNRS, UMR 5665,INRA LA 913, F-69364 Lyon 07, France

[2] Univ Lyon 1, Fac Med Laennec, Lab Histol & Embryol Mol, F-69372 Lyon 08, France

[3] Max Planck Inst Biophys Chem, Dept Mol Cell Biol, D-37077 Gottingen, Germany

来源：

MOLECULAR ENDOCRINOLOGY | 2002年 / 16卷 / 01期

关键词：

D O I：

10.1210/me.16.1.24

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Mice devoid of all TRs are viable, whereas Pax8(-/-)mice, which lack the follicular cells producing T-4 and T-3 in the thyroid gland, die during the first weeks of postnatal life. A precise comparison between the two types of mutants reveals that their phenotypes are similar, but the defects in spleen, bone, and small intestine are more pronounced in Pax(-/-) mice. This is interpreted as the result of a negative effect of the unliganded TR on thyroid hormone target genes expression in the Pax(-/-)mutants. Pax8(-/-) compound mutants can survive to adulthood, and the expression of target genes is partially restored. This demonstrates the importance of TR alpha aporeceptor activity in several aspects of postnatal development.

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页码：24 / 32

页数：9

相关论文

共 57 条

[1] Distinct tissue-specific roles for thyroid hormone receptors β and α1 in regulation of type 1 deiodinase expression [J].

Amma, LL ;

Campos-Barros, A ;

Wang, ZD ;

Vennström, B ;

Forrest, D .

MOLECULAR ENDOCRINOLOGY, 2001, 15 (03) :467-475

[2] Effects of T3Rα1 and T3Rα2 gene deletion on T and B lymphocyte development [J].

Arpin, C ;

Pihlgren, M ;

Fraichard, A ;

Aubert, D ;

Samarut, J ;

Chassande, O ;

Marvel, J .

JOURNAL OF IMMUNOLOGY, 2000, 164 (01) :152-160

[3] Mutations of the human thyrotropin receptor gene causing thyroid hypoplasia and persistent congenital hypothyroidism [J].

Biebermann, H ;

Schoneberg, T ;

Krude, H ;

Schultz, G ;

Gudermann, T ;

Gruters, A .

JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 1997, 82 (10) :3471-3480

[4] Biology of the congenitally hypothyroid hyt/hyt mouse [J].

Biesiada, E ;

Adams, PM ;

Shanklin, DR ;

Bloom, GS ;

Stein, SA .

ADVANCES IN NEUROIMMUNOLOGY, 1996, 6 (04) :309-346

[5] Type 2 iodothyronine deiodinase expression in the cochlea before the onset of hearing [J].

Campos-Barros, A ;

Amma, LL ;

Faris, JS ;

Shailam, R ;

Kelley, MW ;

Forrest, D .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2000, 97 (03) :1287-1292

[6]

CamposBarros A, 1997, J NEUROCHEM, V68, P795

[7] Identification of transcripts initiated from an internal promoter in the c-erbA alpha locus that encode inhibitors of retinoic acid receptor-alpha and triiodothyronine receptor activities [J].

Chassande, O ;

Fraichard, A ;

Gauthier, K ;

Flamant, F ;

Legrand, C ;

Savatier, P ;

Laudet, V ;

Samarut, J .

MOLECULAR ENDOCRINOLOGY, 1997, 11 (09) :1278-1290

[8]

Chassande O, 1999, CURR OPIN ENDOCRINOL, V6, P293

[9] Mutation of the gene encoding human TTF-2 associated with thyroid agenesis, cleft palate and choanal atresia [J].

Clifton-Bligh, RJ ;

Wentworth, JM ;

Heinz, P ;

Crisp, MS ;

John, R ;

Lazarus, JH ;

Ludgate, M ;

Chatterjee, VK .

NATURE GENETICS, 1998, 19 (04) :399-401

[10] Nongenomic actions of thyroid hormone [J].

Davis, PJ ;

Davis, FB .

THYROID, 1996, 6 (05) :497-504

← 1 2 3 4 5 6 →