Heme oxygenase-1 gene promoter polymorphism is associated with reduced incidence of acute chest syndrome among children with sickle cell disease.

Authors: Bean, CJ  Boulet, SL  Ellingsen, D  Pyle, ME  Barron-Casella, EA  Casella, JF  Payne, AB  Driggers, J  Trau, HA  Yang, G  Jones, K  Ofori-Acquah, SF  Hooper, WC  Debaun, MR 
Citation: Bean CJ, etal., Blood. 2012 Nov 1;120(18):3822-8. doi: 10.1182/blood-2011-06-361642. Epub 2012 Sep 10.
Pubmed: (View Article at PubMed) PMID:22966170
DOI: Full-text: DOI:10.1182/blood-2011-06-361642

Sickle cell disease is a common hemolytic disorder with a broad range of complications, including vaso-occlusive episodes, acute chest syndrome (ACS), pain, and stroke. Heme oxygenase-1 (gene HMOX1; protein HO-1) is the inducible, rate-limiting enzyme in the catabolism of heme and might attenuate the severity of outcomes from vaso-occlusive and hemolytic crises. A (GT)(n) dinucleotide repeat located in the promoter region of the HMOX1 gene is highly polymorphic, with long repeat lengths linked to decreased activity and inducibility. We examined this polymorphism to test the hypothesis that short alleles are associated with a decreased risk of adverse outcomes (hospitalization for pain or ACS) among a cohort of 942 children with sickle cell disease. Allele lengths varied from 13 to 45 repeats and showed a trimodal distribution. Compared with children with longer allele lengths, children with 2 shorter alleles (4%;

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CRRD ID: 10755560
Created: 2016-02-01
Species: All species
Last Modified: 2016-02-01
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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.