Identification of a new HBA1 gene mutation (HBA1:c.301-2A>T) in cis with Hb riccarton (HBA1:c.154G>A) [α51(CE9)Gly→Ser]

We report two point mutations found in a heterozygous state on the HBA1 gene of an 88-year-old Argentinean patient with an α+- thalassemia (α+-thal) phenotype: Hb Riccarton HBA1:c.154G>A) [α51(CE9)Gly→Ser] and a novel mutation, HBA1:c.301-2A>T that affects the splicing acceptor site of the sec...

Descripción completa

Detalles Bibliográficos
Autores: Scheps, Karen, Binaghi, Amanda, Varela, Viviana
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2012
País:Argentina
Institución:Consejo Nacional de Investigaciones Científicas y Técnicas
Repositorio:CONICET Digital (CONICET)
Idioma:inglés
OAI Identifier:oai:ri.conicet.gov.ar:11336/67353
Acceso en línea:http://hdl.handle.net/11336/67353
Access Level:acceso abierto
Palabra clave:Α-Thalassemia (Α-Thal)
Genetics
Hba1 Nondeletional Mutation
https://purl.org/becyt/ford/3.3
https://purl.org/becyt/ford/3
Descripción
Sumario:We report two point mutations found in a heterozygous state on the HBA1 gene of an 88-year-old Argentinean patient with an α+- thalassemia (α+-thal) phenotype: Hb Riccarton HBA1:c.154G>A) [α51(CE9)Gly→Ser] and a novel mutation, HBA1:c.301-2A>T that affects the splicing acceptor site of the second intron and leads to a non functional α-globin chain. Cloning of the HBA1 PCR (polymerase chain reaction) product and direct sequencing of the clones revealed that both mutations were in cis.