An intronic SNP in the thyroid hormone receptor β gene is associated with pituitary cell-specific over-expression of a mutant thyroid hormone receptor β2 (R338W) in the index case of pituitary-selective resistance to thyroid hormone
2011

Genetic Factors in Thyroid Hormone Resistance

Sample size: 45 publication Evidence: moderate

Author Information

Author(s): Alberobello Anna Teresa, Congedo Valentina, Liu Hong, Cochran Craig, Skarulis Monica C, Forrest Douglas, Celi Francesco S

Primary Institution: NIDDK-NIH

Hypothesis

Polymorphisms in the human intron control region of the THRB gene could modulate the pituitary expression of the mutated gene contributing to the clinical presentation of resistance to thyroid hormone.

Conclusion

The study illustrates how a combination of a coding mutation and non-coding SNPs can lead to a tissue-specific dominant-negative condition in thyroid hormone resistance.

Supporting Evidence

  • The index case of pituitary-selective resistance is characterized by the missense R338W mutation in cis with two common SNPs.
  • Reporter gene assays indicated that the rs2596623T SNP increases pituitary cell-specific activity of the TR β2 promoter.
  • The study found that the combined coding mutation and non-coding SNPs create a dominant-negative condition affecting thyroid hormone response.

Takeaway

Some people have a condition where their body doesn't respond properly to thyroid hormone, and this study found that specific genetic changes can make this problem worse.

Methodology

Screening and in vitro characterization of polymorphisms of the intron enhancer region of the THRB gene.

Limitations

The study's findings may not be generalizable due to the small sample size and the lack of genetic material from relatives for haplotype determination.

Participant Demographics

The study involved 45 patients with resistance to thyroid hormone, categorized as either generalized or pituitary-selective resistance.

Statistical Information

P-Value

p<0.0001

Statistical Significance

p<0.0001

Digital Object Identifier (DOI)

10.1186/1479-5876-9-144

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