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Thyroid hormone receptor beta: Wikis


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Thyroid hormone receptor, beta (erythroblastic leukemia viral (v-erb-a) oncogene homolog 2, avian)

PDB rendering based on 1bsx.
Available structures
1bsx, 1n46, 1nax, 1nq0, 1nq1, 1nq2, 1nuo, 1q4x, 1r6g, 1xzx, 1y0x, 2h6w, 2nll
Symbols THRB; ERBA-BETA; ERBA2; GRTH; MGC126109; MGC126110; NR1A2; THR1; THRB1; THRB2
External IDs OMIM190160 MGI98743 HomoloGene36025 GeneCards: THRB Gene
RNA expression pattern
PBB GE THRB 207044 at tn.png
More reference expression data
Species Human Mouse
Entrez 7068 21834
Ensembl ENSG00000151090 ENSMUSG00000021779
UniProt P10828 Q0VDR8
RefSeq (mRNA) NM_000461 NM_009380
RefSeq (protein) NP_000452 NP_033406
Location (UCSC) Chr 3:
24.13 - 24.51 Mb
Chr 14:
16.76 - 16.83 Mb
PubMed search [1] [2]

Thyroid hormone receptor beta (TR-beta) (erythroblastic leukemia viral (v-erb-a) oncogene homolog 2, avian), also known as NR1A2 (nuclear receptor subfamily 1, group A, member 2), is nuclear receptor protein encoded by the THRB gene.[1]

The protein encoded by this gene is a nuclear hormone receptor for triiodothyronine. It is one of the several receptors for thyroid hormone, and has been shown to mediate the biological activities of thyroid hormone. Knockout studies in mice suggest that the different receptors, while having certain extent of redundancy, may mediate different functions of thyroid hormone. Defects in this gene are known to be a cause of generalized thyroid hormone resistance (GTHR), a syndrome characterized by goiter and high levels of circulating thyroid hormone (T3-T4), with normal or slightly elevated thyroid stimulating hormone (TSH). Several transcript variants have been observed for this gene, but the full-length nature of only one has been observed so far.[1]


Thyroid hormone receptor beta has been shown to interact with NCOA6,[2][3][4][5] PPARGC1A,[6] Nuclear receptor coactivator 1,[7][8] Nuclear receptor co-repressor 2,[7][9][10] Cyclin D1,[11] V-erbA-related gene,[12] BRD8[13] and Retinoid X receptor alpha.[13][8]


  1. ^ a b "Entrez Gene: THRA thyroid hormone receptor, alpha (erythroblastic leukemia viral (v-erb-a) oncogene homolog, avian)".  
  2. ^ Lee, S K; Jung S Y, Kim Y S, Na S Y, Lee Y C, Lee J W (Feb. 2001). "Two distinct nuclear receptor-interaction domains and CREB-binding protein-dependent transactivation function of activating signal cointegrator-2". Mol. Endocrinol. (United States) 15 (2): 241–54. ISSN 0888-8809. PMID 11158331.  
  3. ^ Lee, S K; Anzick S L, Choi J E, Bubendorf L, Guan X Y, Jung Y K, Kallioniemi O P, Kononen J, Trent J M, Azorsa D, Jhun B H, Cheong J H, Lee Y C, Meltzer P S, Lee J W (Nov. 1999). "A nuclear factor, ASC-2, as a cancer-amplified transcriptional coactivator essential for ligand-dependent transactivation by nuclear receptors in vivo". J. Biol. Chem. (UNITED STATES) 274 (48): 34283–93. ISSN 0021-9258. PMID 10567404.  
  4. ^ Ko, L; Cardona G R, Chin W W (May. 2000). "Thyroid hormone receptor-binding protein, an LXXLL motif-containing protein, functions as a general coactivator". Proc. Natl. Acad. Sci. U.S.A. (UNITED STATES) 97 (11): 6212–7. ISSN 0027-8424. PMID 10823961.  
  5. ^ Ko, Lan; Cardona Guemalli R, Iwasaki Toshiharu, Bramlett Kelli S, Burris Thomas P, Chin William W (Jan. 2002). "Ser-884 adjacent to the LXXLL motif of coactivator TRBP defines selectivity for ERs and TRs". Mol. Endocrinol. (United States) 16 (1): 128–40. ISSN 0888-8809. PMID 11773444.  
  6. ^ Wu, Yifei; Delerive Philippe, Chin William W, Burris Thomas P (Mar. 2002). "Requirement of helix 1 and the AF-2 domain of the thyroid hormone receptor for coactivation by PGC-1". J. Biol. Chem. (United States) 277 (11): 8898–905. doi:10.1074/jbc.M110761200. ISSN 0021-9258. PMID 11751919.  
  7. ^ a b Liu, Y; Takeshita A, Misiti S, Chin W W, Yen P M (Oct. 1998). "Lack of coactivator interaction can be a mechanism for dominant negative activity by mutant thyroid hormone receptors". Endocrinology (UNITED STATES) 139 (10): 4197–204. ISSN 0013-7227. PMID 9751500.  
  8. ^ a b Jeyakumar, M; Tanen M R, Bagchi M K (Jun. 1997). "Analysis of the functional role of steroid receptor coactivator-1 in ligand-induced transactivation by thyroid hormone receptor". Mol. Endocrinol. (UNITED STATES) 11 (6): 755–67. ISSN 0888-8809. PMID 9171239.  
  9. ^ Tagami, T; Lutz W H, Kumar R, Jameson J L (Dec. 1998). "The interaction of the vitamin D receptor with nuclear receptor corepressors and coactivators". Biochem. Biophys. Res. Commun. (UNITED STATES) 253 (2): 358–63. doi:10.1006/bbrc.1998.9799. ISSN 0006-291X. PMID 9878542.  
  10. ^ Ando, S; Sarlis N J, Krishnan J, Feng X, Refetoff S, Zhang M Q, Oldfield E H, Yen P M (Sep. 2001). "Aberrant alternative splicing of thyroid hormone receptor in a TSH-secreting pituitary tumor is a mechanism for hormone resistance". Mol. Endocrinol. (United States) 15 (9): 1529–38. ISSN 0888-8809. PMID 11518802.  
  11. ^ Lin, Huei-Min; Zhao Li, Cheng Sheue-Yann (Aug. 2002). "Cyclin D1 Is a Ligand-independent Co-repressor for Thyroid Hormone Receptors". J. Biol. Chem. (United States) 277 (32): 28733–41. doi:10.1074/jbc.M203380200. ISSN 0021-9258. PMID 12048199.  
  12. ^ Zhu, X G; Park K S, Kaneshige M, Bhat M K, Zhu Q, Mariash C N, McPhie P, Cheng S Y (Apr. 2000). "The orphan nuclear receptor Ear-2 is a negative coregulator for thyroid hormone nuclear receptor function". Mol. Cell. Biol. (UNITED STATES) 20 (7): 2604–18. ISSN 0270-7306. PMID 10713182.  
  13. ^ a b Monden, T; Wondisford F E, Hollenberg A N (Nov. 1997). "Isolation and characterization of a novel ligand-dependent thyroid hormone receptor-coactivating protein". J. Biol. Chem. (UNITED STATES) 272 (47): 29834–41. ISSN 0021-9258. PMID 9368056.  

Further reading

  • Lazar MA, Chin WW (1991). "Nuclear thyroid hormone receptors.". J. Clin. Invest. 86 (6): 1777–82. doi:10.1172/JCI114906. PMID 2254444.  
  • Forrest D, Reh TA, Rüsch A (2002). "Neurodevelopmental control by thyroid hormone receptors.". Curr. Opin. Neurobiol. 12 (1): 49–56. doi:10.1016/S0959-4388(02)00289-1. PMID 11861164.  
  • González-Sancho JM, García V, Bonilla F, Muñoz A (2003). "Thyroid hormone receptors/THR genes in human cancer.". Cancer Lett. 192 (2): 121–32. doi:10.1016/S0304-3835(02)00614-6. PMID 12668276.  
  • Cugini CD, Leidy JW, Chertow BS, et al. (1992). "An arginine to histidine mutation in codon 315 of the c-erbA beta thyroid hormone receptor in a kindred with generalized resistance to thyroid hormones results in a receptor with significant 3,5,3'-triiodothyronine binding activity.". J. Clin. Endocrinol. Metab. 74 (5): 1164–70. doi:10.1210/jc.74.5.1164. PMID 1314846.  
  • Behr M, Loos U (1992). "A point mutation (Ala229 to Thr) in the hinge domain of the c-erbA beta thyroid hormone receptor gene in a family with generalized thyroid hormone resistance.". Mol. Endocrinol. 6 (7): 1119–26. doi:10.1210/me.6.7.1119. PMID 1324420.  
  • Falcone M, Miyamoto T, Fierro-Renoy F, et al. (1992). "Antipeptide polyclonal antibodies specifically recognize each human thyroid hormone receptor isoform.". Endocrinology 131 (5): 2419–29. doi:10.1210/en.131.5.2419. PMID 1425440.  
  • Schmidt ED, Schmidt ED, van der Gaag R, et al. (1992). "Distribution of the nuclear thyroid-hormone receptor in extraocular and skeletal muscles.". J. Endocrinol. 133 (1): 67–74. doi:10.1677/joe.0.1330067. PMID 1517709.  
  • Adams M, Nagaya T, Tone Y, et al. (1992). "Functional properties of a novel mutant thyroid hormone receptor in a family with generalized thyroid hormone resistance syndrome.". Clin. Endocrinol. (Oxf) 36 (3): 281–9. doi:10.1111/j.1365-2265.1992.tb01444.x. PMID 1563081.  
  • Sasaki S, Nakamura H, Tagami T, et al. (1992). "A point mutation of the T3 receptor beta 1 gene in a kindred of generalized resistance to thyroid hormone.". Mol. Cell. Endocrinol. 84 (3): 159–66. doi:10.1016/0303-7207(92)90026-3. PMID 1587388.  
  • Nagaya T, Madison LD, Jameson JL (1992). "Thyroid hormone receptor mutants that cause resistance to thyroid hormone. Evidence for receptor competition for DNA sequences in target genes.". J. Biol. Chem. 267 (18): 13014–9. PMID 1618799.  
  • Shuto Y, Wakabayashi I, Amuro N, et al. (1992). "A point mutation in the 3,5,3'-triiodothyronine-binding domain of thyroid hormone receptor-beta associated with a family with generalized resistance to thyroid hormone.". J. Clin. Endocrinol. Metab. 75 (1): 213–7. doi:10.1210/jc.75.1.213. PMID 1619012.  
  • Näär AM, Boutin JM, Lipkin SM, et al. (1991). "The orientation and spacing of core DNA-binding motifs dictate selective transcriptional responses to three nuclear receptors.". Cell 65 (7): 1267–79. doi:10.1016/0092-8674(91)90021-P. PMID 1648451.  
  • Usala SJ, Menke JB, Watson TL, et al. (1991). "A homozygous deletion in the c-erbA beta thyroid hormone receptor gene in a patient with generalized thyroid hormone resistance: isolation and characterization of the mutant receptor.". Mol. Endocrinol. 5 (3): 327–35. doi:10.1210/mend-5-3-327. PMID 1653889.  
  • Bodenner DL, Mroczynski MA, Weintraub BD, et al. (1991). "A detailed functional and structural analysis of a major thyroid hormone inhibitory element in the human thyrotropin beta-subunit gene.". J. Biol. Chem. 266 (32): 21666–73. PMID 1657975.  
  • Parrilla R, Mixson AJ, McPherson JA, et al. (1992). "Characterization of seven novel mutations of the c-erbA beta gene in unrelated kindreds with generalized thyroid hormone resistance. Evidence for two "hot spot" regions of the ligand binding domain.". J. Clin. Invest. 88 (6): 2123–30. doi:10.1172/JCI115542. PMID 1661299.  
  • Usala SJ, Menke JB, Watson TL, et al. (1991). "A new point mutation in the 3,5,3'-triiodothyronine-binding domain of the c-erbA beta thyroid hormone receptor is tightly linked to generalized thyroid hormone resistance.". J. Clin. Endocrinol. Metab. 72 (1): 32–8. PMID 1846005.  
  • Sakurai A, Nakai A, DeGroot LJ (1990). "Structural analysis of human thyroid hormone receptor beta gene.". Mol. Cell. Endocrinol. 71 (2): 83–91. doi:10.1016/0303-7207(90)90245-4. PMID 1973914.  
  • Usala SJ, Tennyson GE, Bale AE, et al. (1990). "A base mutation of the C-erbA beta thyroid hormone receptor in a kindred with generalized thyroid hormone resistance. Molecular heterogeneity in two other kindreds.". J. Clin. Invest. 85 (1): 93–100. doi:10.1172/JCI114438. PMID 2153155.  
  • Sakurai A, Takeda K, Ain K, et al. (1989). "Generalized resistance to thyroid hormone associated with a mutation in the ligand-binding domain of the human thyroid hormone receptor beta.". Proc. Natl. Acad. Sci. U.S.A. 86 (22): 8977–81. doi:10.1073/pnas.86.22.8977. PMID 2510172.  

This article incorporates text from the United States National Library of Medicine, which is in the public domain.



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