| http://purl.uniprot.org/citations/15545996 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/15545996 | http://www.w3.org/2000/01/rdf-schema#comment | "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."xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.org/dc/terms/identifier | "doi:10.1172/jci21982"xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/author | "Yu X."xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/author | "Zhou S."xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/author | "Kong Y."xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/author | "Adachi K."xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/author | "Weiss M.J."xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/author | "Mackay J.P."xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/author | "Gell D.A."xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/author | "Gow A.J."xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/author | "Kihm A.J."xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/author | "Louden C.S."xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/author | "Foster-Brown L."xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/author | "Katein A.M."xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/date | "2004"xsd:gYear |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/name | "J Clin Invest"xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/pages | "1457-1466"xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/title | "Loss of alpha-hemoglobin-stabilizing protein impairs erythropoiesis and exacerbates beta-thalassemia."xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://purl.uniprot.org/core/volume | "114"xsd:string |
| http://purl.uniprot.org/citations/15545996 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/15545996 |
| http://purl.uniprot.org/citations/15545996 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/15545996 |
| http://purl.uniprot.org/uniprot/Q03963#attribution-3310A3351EDF3961509956FBAC64A9ED | http://purl.uniprot.org/core/source | http://purl.uniprot.org/citations/15545996 |
| http://purl.uniprot.org/uniprot/#_P02088-mappedCitation-15545996 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/15545996 |
| http://purl.uniprot.org/uniprot/#_P02089-mappedCitation-15545996 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/15545996 |