Ontology highlight
ABSTRACT:
SUBMITTER: Nasimuzzaman M
PROVIDER: S-EPMC3632304 | biostudies-literature | 2010 Oct
REPOSITORIES: biostudies-literature
Nasimuzzaman Md M Khandros Eugene E Wang Xiaomei X Kong Yi Y Zhao Huifen H Weiss David D Rivella Stefano S Weiss Mitchell J MJ Persons Derek A DA
American journal of hematology 20101001 10
Excess free alpha-globin is cytotoxic and contributes to the pathophysiology of b-thalassemia. Alpha hemoglobin stabilizing protein (AHSP) is a molecular chaperone that binds free alpha-globin to promote its folding and inhibit its ability to produce damaging reactive oxygen species. Reduced AHSP levels correlate with increased severity of b-thalassemia in some human cohorts, but causal mechanistic relationships are not established for these associations. We used transgenic and lentiviral gene t ...[more]