A four-year-old girl with pathogenic variant in the NAA10 gene and precocious puberty - case report and literature review

Ann Agric Environ Med. 2024 Jun 27;31(2):306-310. doi: 10.26444/aaem/171758. Epub 2023 Sep 22.

Abstract

The NAA10 gene encodes N-alpha-acetyltransferase 10 which plays an important role in cell growth, differentiation, DNA damage, metastasis, apoptosis, stress response and autophagy. Defects in the NAA10 gene correlate with the diagnosis of NAA10-related syndrome (Ogden syndrome). The most common symptoms of NAA10-related syndrome are: global developmental delay, non-verbal or limited speech, autism spectrum disorder, feeding difficulties, motor delay, muscle tone disturbances, and long QT syndrome. To-date, there are about 100 patients who have been reported with this condition. The case report presents the clinical study of a girl aged 4 years and 3 months diagnosed with Ogden syndrome. She had many characteristic features of the disorder, as well as precocious puberty. This girl represents the case of a patient with p.Arg83Cys mutation in NAA10 gene as well as precocious puberty.

Keywords: NAA10 gene; NAA10-related syndrome; Ogden syndrome; precocious puberty.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Child, Preschool
  • Female
  • Humans
  • Mutation
  • N-Terminal Acetyltransferase A* / genetics
  • N-Terminal Acetyltransferase A* / metabolism
  • N-Terminal Acetyltransferase E* / genetics
  • N-Terminal Acetyltransferase E* / metabolism
  • Puberty, Precocious* / genetics

Substances

  • N-Terminal Acetyltransferase A
  • N-Terminal Acetyltransferase E
  • NAA10 protein, human