AI Article Synopsis

  • The report highlights the activities of the Molecular Genetics-Function and Therapy (MGFT) department at the Cyprus Institute of Neurology and Genetics, emphasizing its role as a Reference Center for the European Network on Rare Endocrine Conditions (Endo-ERN).
  • Over 15 years of collaboration has resulted in more than 2000 genetic tests performed for diagnosing rare inherited endocrine disorders, with significant findings related to various genetic variants associated with conditions like Congenital Adrenal Hyperplasia (CAH) and Multiple Endocrine Neoplasia (MEN).
  • The MGFT has also developed a diagnostic and research program focusing on premature puberty, particularly the role of the MKRN3 gene, aiming to enhance healthcare for patients with rare endocrine

Article Abstract

The report covers the current and past activities of the department Molecular Genetics-Function and Therapy (MGFT) at the Cyprus Institute of Neurology and Genetics (CING), an affiliated Reference Center for the European Reference Network on Rare Endocrine Conditions (Endo-ERN).The presented data is the outcome of > 15 years long standing collaboration between MGFT and endocrine specialists from the local government hospitals and the private sector. Up-to-date > 2000 genetic tests have been performed for the diagnosis of inherited rare endocrine disorders. The major clinical entities included Congenital Adrenal Hyperplasia (CAH) due to pathogenic variants in CYP21A2 gene and Multiple Endocrine Neoplasia (MEN) type 2 due to pathogenic variants in the RET proto-oncogene. Other rare and novel pathogenic variants in ANOS1, WDR11, FGFR1, RNF216, and CHD7 genes were also found in patients with Congenital Hypogonadotropic Hypogonadism. Interestingly, a few patients with Disorders of Sexual Differentiation (DSD) shared rare pathogenic variants in the SRD5A2, HSD17B3 and HSD3B2 while patients with Glucose and Insulin Homeostasis carried theirs in GCK and HNF1A genes. Lastly, MGFT over the last few years has established an esteemed diagnostic and research program on premature puberty with emphasis on the implication of MKRN3 gene on the onset of the disease and the identification of other prognosis biomarkers.As an Endo-ERN member MGFT department belongs to this large European network and holds the same humanistic ideals which aim toward the improvements of health care for patients with rare endocrine conditions in respect to improved and faster diagnosis.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11027394PMC
http://dx.doi.org/10.1186/s13023-024-03171-4DOI Listing

Publication Analysis

Top Keywords

pathogenic variants
16
rare endocrine
12
endocrine disorders
8
cyprus institute
8
institute neurology
8
neurology genetics
8
reference center
8
endocrine conditions
8
endocrine
6
rare
5

Similar Publications

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!