Literature DB >> 22857144

Overview of genetic defects in endocrinopathies in the island of Cyprus; evidence of a founder effect.

Christos Shammas1, Vassos Neocleous, Meropi Toumba, Constantina Costi, Alexia A P Phedonos, Elisavet Efstathiou, Andreas Kyriakou, Leonidas A Phylactou, Nicos Skordis.   

Abstract

AIM: Hereditary endocrinopathies in Cyprus exhibit evidence of a founder effect and display the influence of past migration patterns. The genetic frequency and mutation pattern of a specific disorder of sex development (DSD), which is classified as 46,XX DSD or 46,XY DSD, and the non-classic form of congenital adrenal hyperplasia (NC-CAH) outline a type of genetic drift.
RESULTS: Not only the high prevalence of the NC-CAH p.V281L mutation but also the rarity of CAH large lesions present a genetic diversity similar to that observed in the Middle Eastern countries. In addition, both the high frequency of the 5-alpha steroid reductase deficiency (5αSRD) IVS1-2A>G mutation and the carrier frequency of the 17-beta hydroxysteroid dehydrogenase 3 (17β-HSD-3) p.R80Q mutation are good examples of a founder effect. p.R80Q can be considered a founder mutation, even though it has been identified in patients of Dutch, Brazilian, and Portuguese origin. This has led to the speculation that it has a Phoenician origin. Phoenicians as ancient traders migrated around 750 BC from present day Syria, Lebanon, and Israel toward Portugal, Spain, and also to nearby Cyprus. While the 5αSRD IVS1-2A>G mutation has already been extensively reported in Turkish patients, it is very common in the Eastern Mediterranean region.
CONCLUSION: This short article portrays clearly, through specific endocrine genetic disorders, the past migration trends in Cyprus that shaped the present-day gene pool of the Greek-Cypriot population.

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Year:  2012        PMID: 22857144     DOI: 10.1089/gtmb.2011.0381

Source DB:  PubMed          Journal:  Genet Test Mol Biomarkers        ISSN: 1945-0257


  7 in total

1.  Molecular genetics and phenotype/genotype correlation of 5-α reductase deficiency in a highly consanguineous population.

Authors:  Meshael M Alswailem; Ohoud S Alzahrani; Lamyaa Alghofaili; Ebtesam Qasem; Mai Almohanaa; Afaf Alsagheir; Bassam Bin Abbas; Najya A Attia; Adnan Al Shaikh; Ali S Alzahrani
Journal:  Endocrine       Date:  2018-09-29       Impact factor: 3.633

2.  A Novel Mutation Causing 17-β-Hydroxysteroid Dehydrogenase Type 3 Deficiency in an Omani Child: First Case Report and Review of Literature.

Authors:  Aisha Al-Sinani; Waad-Allah S Mula-Abed; Manal Al-Kindi; Ghariba Al-Kusaibi; Hanan Al-Azkawi; Nahid Nahavandi
Journal:  Oman Med J       Date:  2015-03

3.  Genetic defects of the CYP21A2 gene in girls with premature adrenarche.

Authors:  N Skordis; C Shammas; A A P Phedonos; A Kyriakou; M Toumba; V Neocleous; L A Phylactou
Journal:  J Endocrinol Invest       Date:  2014-12-07       Impact factor: 4.256

4.  Phenotypic variability of hyperandrogenemia in females heterozygous for CYP21A2 mutations.

Authors:  Vassos Neocleous; Christos Shammas; Alexia Ap Phedonos; Leonidas A Phylactou; Nicos Skordis
Journal:  Indian J Endocrinol Metab       Date:  2014-11

5.  46,XY Disorder of Sex Development due to 17-Beta Hydroxysteroid Dehydrogenase Type 3 Deficiency in an Infant of Greek Origin.

Authors:  Assimina Galli-Tsinopoulou; Anastasios Serbis; Eleni P Kotanidou; Eleni Litou; Vaia Dokousli; Konstantina Mouzaki; Pavlos Fanis; Vassos Neocleous; Nicos Skordis
Journal:  J Clin Res Pediatr Endocrinol       Date:  2017-07-24

6.  GnRH Deficient Patients With Congenital Hypogonadotropic Hypogonadism: Novel Genetic Findings in ANOS1, RNF216, WDR11, FGFR1, CHD7, and POLR3A Genes in a Case Series and Review of the Literature.

Authors:  Vassos Neocleous; Pavlos Fanis; Meropi Toumba; George A Tanteles; Melpo Schiza; Feride Cinarli; Nicolas C Nicolaides; Anastasis Oulas; George M Spyrou; Christos S Mantzoros; Dimitrios Vlachakis; Nicos Skordis; Leonidas A Phylactou
Journal:  Front Endocrinol (Lausanne)       Date:  2020-08-28       Impact factor: 5.555

7.  Multiple endocrine neoplasia 2 in Cyprus: evidence for a founder effect.

Authors:  P Fanis; N Skordis; S Frangos; G Christopoulos; E Spanou-Aristidou; E Andreou; P Manoli; M Mavrommatis; S Nicolaou; M Kleanthous; M A Cariolou; V Christophidou-Anastasiadou; G A Tanteles; L A Phylactou; V Neocleous
Journal:  J Endocrinol Invest       Date:  2018-02-02       Impact factor: 4.256

  7 in total

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