Severe forms of partial androgen insensitivity syndrome due to p.L830F novel mutation in androgen receptor gene in a Brazilian family
2011

New Mutation in Androgen Receptor Linked to Androgen Insensitivity Syndrome in Brazilian Family

Sample size: 7 publication Evidence: moderate

Author Information

Author(s): Petroli Reginaldo J, Maciel-Guerra Andréa T, Soardi Fernanda C, de Calais Flávia L, Guerra-Junior Gil, de Mello Maricilda Palandi

Primary Institution: Universidade de Campinas (UNICAMP)

Hypothesis

The study aims to identify the AR gene mutation in a Brazilian family with partial androgen insensitivity syndrome.

Conclusion

The novel p.L830F mutation is responsible for grades 5 and 6 of partial androgen insensitivity syndrome in two generations of a Brazilian family.

Supporting Evidence

  • The mutation was identified in five 46,XY female hemizygote individuals and two heterozygote carrier mothers.
  • The study provides important information for the function of a specific amino acid residue in the androgen receptor.
  • The presence of the mutation was linked to different grades of partial androgen insensitivity syndrome in family members.

Takeaway

This study found a new mutation in a gene that affects how the body responds to male hormones, which can cause problems with sexual development.

Methodology

The study sequenced the coding exons and exon-intron junctions of the androgen receptor gene in seven family members.

Participant Demographics

The study included five 46,XY females and two healthy mothers from a Brazilian family.

Digital Object Identifier (DOI)

10.1186/1756-0500-4-173

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