Mitochondrial Changes in β0-Thalassemia/Hb E Disease.

ABSTRACT: The compound β°-thalassemia/Hb E hemoglobinopathy is characterized by an unusually large range of presentation from essentially asymptomatic to a severe transfusion dependent state. While a number of factors are known that moderate presentation, these factors do not account for the full spectrum of presentation. Mitochondria are subcellular organelles that are pivotal in a number of cellular processes including oxidative phosphorylation and apoptosis. A mitochondrial protein enriched proteome was determined and validated from erythroblasts from normal controls and β°-thalassemia/Hb E patients of different severities. Mitochondria were evaluated through the use of mitotracker staining, analysis of relative mitochondrial genome number and evaluation of mitochondrial gene expression in additi

SUBMITTER: Khungwanmaythawee K 

PROVIDER: S-EPMC4836671 | biostudies-literature | 2016

REPOSITORIES: biostudies-literature