| Literature DB >> 34435747 |
Emily Mira Warshauer1,2, Adam Brown3, Ignacia Fuentes4,5, Jonathan Shortt6, Chris Gignoux6, Francesco Montinaro7,8, Mait Metspalu7, Leila Youssefian9, Hassan Vahidnezhad9, Joanna Jacków10,11, Angela M Christiano10,12, Jouni Uitto9, Óscar R Fajardo-Ramírez13,14, Julio C Salas-Alanis13,15, John A McGrath11, Liliana Consuegra16, Carolina Rivera16,17, Paul A Maier18, Goran Runfeldt18, Doron M Behar7,18, Karl Skorecki19, Eli Sprecher20,21, Francis Palisson5,22, David A Norris1,2, Anna L Bruckner1, Igor Kogut1,2, Ganna Bilousova1,2, Dennis R Roop1,2.
Abstract
Recessive dystrophic epidermolysis bullosa (RDEB) is a rare genodermatosis caused by mutations in the gene coding for type VII collagen (COL7A1). More than 800 different pathogenic mutations in COL7A1 have been described to date; however, the ancestral origins of many of these mutations have not been precisely identified. In this study, 32 RDEB patient samples from the Southwestern United States, Mexico, Chile, and Colombia carrying common mutations in the COL7A1 gene were investigated to determine the origins of these mutations and the extent to which shared ancestry contributes to disease prevalence. The results demonstrate both shared European and American origins of RDEB mutations in distinct populations in the Americas and suggest the influence of Sephardic ancestry in at least some RDEB mutations of European origins. Knowledge of ancestry and relatedness among RDEB patient populations will be crucial for the development of future clinical trials and the advancement of novel therapeutics.Entities:
Keywords: epidermolysis bullosa; genetics; genodermatoses
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Year: 2021 PMID: 34435747 PMCID: PMC8668271 DOI: 10.1002/ajmg.a.62456
Source DB: PubMed Journal: Am J Med Genet A ISSN: 1552-4825 Impact factor: 2.802