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NR0B1

MIM.300473 Xp21.3-p21.2

Pathology

- germline mutations in

  • congenital adrenal hypoplasia (AHC) (MIM.300200)
    • and hypogonadotropic hypogonadism
    • and bilateral cryptorchidism
  • dosage sensitive male-to-female sex reversal (MIM.300018)
  • delayed-onset adrenal insufficiency and hypogonadotrophic hypogonadism

References

- Ludbrook LM, Harley VR. Sex determination: a ’window’ of DAX1 activity. Trends Endocrinol Metab. 2004 Apr;15(3):116-21. PMID: 15046740