Literature DB >> 7795641

Sequence analysis reveals a beta-thalassaemia mutation in the DNA of skeletal remains from the archaeological site of Akhziv, Israel.

D Filon1, M Faerman, P Smith, A Oppenheim.   

Abstract

beta-Thalassaemia is manifested by severe anaemia and extensive bone pathology. Similar pathology may also result from other forms of anaemia. To clarify the precise cause, we performed DNA analyses on archaeological remains of a child with severe bone pathology. We found homozygosity for frameshift in codon 8 of beta-globin, causing a beta-null phenotype. Paradoxically, the child died when eight years old, whereas such patients are transfusion dependent from early infancy. An infrequent polymorphic marker in the child's DNA, and information from present-day patients, indicated that amelioration of the clinical condition was due to elevated fetal haemoglobin production. Thus this analysis provided not only precise diagnosis of a genetic disease but also allowed clarification of the molecular mechanism underlying the clinical presentation.

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Year:  1995        PMID: 7795641     DOI: 10.1038/ng0495-365

Source DB:  PubMed          Journal:  Nat Genet        ISSN: 1061-4036            Impact factor:   38.330


  3 in total

1.  Postmortem molecular diagnosis of sickle beta thalassaemia.

Authors:  F Kutlar; D Mirmow; M Glendenning; L Holley; A Kutlar
Journal:  J Clin Pathol       Date:  2005-05       Impact factor: 3.411

Review 2.  Archaeogenetics in evolutionary medicine.

Authors:  Abigail Bouwman; Frank Rühli
Journal:  J Mol Med (Berl)       Date:  2016-06-11       Impact factor: 4.599

Review 3.  β-Thalassemia Distribution in the Old World: an Ancient Disease Seen from a Historical Standpoint.

Authors:  Vincenzo De Sanctis; Christos Kattamis; Duran Canatan; Ashraf T Soliman; Heba Elsedfy; Mehran Karimi; Shahina Daar; Yasser Wali; Mohamed Yassin; Nada Soliman; Praveen Sobti; Soad Al Jaouni; Mohamed El Kholy; Bernadette Fiscina; Michael Angastiniotis
Journal:  Mediterr J Hematol Infect Dis       Date:  2017-02-20       Impact factor: 2.576

  3 in total

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