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@article{
 title = {Loss of α-hemoglobin–stabilizing protein impairs erythropoiesis and exacerbates β-thalassemia},
 type = {article},
 year = {2004},
 identifiers = {[object Object]},
 pages = {1457-1466},
 volume = {114},
 websites = {http://www.ncbi.nlm.nih.gov/pubmed/15545996},
 month = {11},
 day = {15},
 id = {bd20b865-7be1-37cb-ab91-c4c1262f5c3b},
 created = {2020-12-17T05:29:56.940Z},
 file_attached = {false},
 profile_id = {a5a2ab6f-a8b5-3db6-97bc-618752ee4386},
 group_id = {bc1ab1d4-9e57-37e6-9fb5-435fca0ee9d2},
 last_modified = {2020-12-17T05:29:56.940Z},
 read = {false},
 starred = {false},
 authored = {false},
 confirmed = {true},
 hidden = {false},
 citation_key = {Kong2004},
 source_type = {ARTICLE},
 notes = {cited By 110},
 private_publication = {false},
 abstract = {Hemoglobin (Hb) A production during red blood cell development is coordinated to minimize the deleterious effects of free alpha- and beta-Hb subunits, which are unstable and cytotoxic. The alpha-Hb-stabilizing protein (AHSP) is an erythroid protein that specifically binds alpha-Hb and prevents its precipitation in vitro, which suggests that it may function to limit free alpha-Hb toxicities in vivo. We investigated this possibility through gene ablation and biochemical studies. AHSP(-/-) erythrocytes contained hemoglobin precipitates and were short-lived. In hematopoietic tissues, erythroid precursors were elevated in number but exhibited increased apoptosis. Consistent with unstable alpha-Hb, AHSP(-/-) erythrocytes contained increased ROS and evidence of oxidative damage. Moreover, purified recombinant AHSP inhibited ROS production by alpha-Hb in solution. Finally, loss of AHSP worsened the phenotype of beta-thalassemia, a common inherited anemia characterized by excess free alpha-Hb. Together, the data support a model in which AHSP binds alpha-Hb transiently to stabilize its conformation and render it biochemically inert prior to Hb A assembly. This function is essential for normal erythropoiesis and, to a greater extent, in beta-thalassemia. Our findings raise the possibility that altered AHSP expression levels could modulate the severity of beta-thalassemia in humans.},
 bibtype = {article},
 author = {Kong, Yi and Zhou, Suiping and Kihm, Anthony J. and Katein, Anne M. and Yu, Xiang and Gell, David A. and Mackay, Joel P. and Adachi, Kazuhiko and Foster-Brown, Linda and Louden, Calvert S. and Gow, Andrew J. and Weiss, Mitchell J.},
 journal = {Journal of Clinical Investigation},
 number = {10}
}