Aberrant Epigenetic Alteration of PAX1 Expression Contributes to Parathyroid Tumorigenesis

J Clin Endocrinol Metab. 2022 Jan 18;107(2):e783-e792. doi: 10.1210/clinem/dgab626.

Abstract

Context: Primary hyperparathyroidism (PHPT) results from the hypersecretion of parathyroid hormone from parathyroid tumors. A transcription factor, namely Paired box1 (PAX1), is active in parathyroid gland development.

Objective: We aimed to study potential epigenetic-mediated mechanism of PAX1 gene in sporadic parathyroid adenomas.

Methods: In parathyroid adenomas tissues, we analyzed the DNA methylation via bisulfite-specific polymerase chain reaction (BSP) and histone modifications via chromatin immunoprecipitation in regulating the differential expression of PAX1.

Results: The results showed that mRNA and protein expression of PAX1 was significantly reduced in parathyroid adenomas. Bisulfite sequencing demonstrated hypermethylation in the promoter region of PAX1 (35%; 14/40) and lower levels of histone 3 lysine 9 acetylation (H3K9ac) were observed on the promoter region of PAX1 (6-fold; P < .004) in parathyroid adenomas. Furthermore, upon treatment with a pharmacologic inhibitor, namely 5'aza-2 deoxycytidine, in rat parathyroid continuous cells, we found re-expression of PAX1 gene.

Conclusion: Our study not only reveals expression of PAX1 is epigenetically deregulated but also paves a way for clinical and therapeutic implications in patients with PHPT.

Publication types

  • Observational Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acetylation / drug effects
  • Adenoma / genetics*
  • Adenoma / pathology
  • Adenoma / therapy
  • Adolescent
  • Adult
  • Aged
  • Animals
  • Carcinogenesis / drug effects
  • Carcinogenesis / genetics*
  • Case-Control Studies
  • Cell Line, Tumor
  • DNA Methylation / drug effects
  • Decitabine / pharmacology
  • Decitabine / therapeutic use
  • Epigenesis, Genetic / drug effects
  • Epigenesis, Genetic / genetics*
  • Female
  • Gene Expression Regulation, Neoplastic / drug effects
  • Healthy Volunteers
  • Histone Code / drug effects
  • Humans
  • Hydroxamic Acids / pharmacology
  • Hydroxamic Acids / therapeutic use
  • Male
  • Middle Aged
  • Paired Box Transcription Factors / genetics*
  • Paired Box Transcription Factors / metabolism
  • Parathyroid Glands / pathology
  • Parathyroid Glands / surgery
  • Parathyroid Neoplasms / genetics*
  • Parathyroid Neoplasms / pathology
  • Parathyroid Neoplasms / therapy
  • Parathyroidectomy
  • Promoter Regions, Genetic / genetics
  • Rats
  • Young Adult

Substances

  • Hydroxamic Acids
  • Paired Box Transcription Factors
  • PAX1 transcription factor
  • trichostatin A
  • Decitabine