Article
Human heme oxygenase-1 deficiency presenting with hemolysis, nephritis, and asplenia.
Journal of pediatric hematology/oncology - 1 Jan 2011
Radhakrishnan Nita, Yadav Satya Prakash, Sachdeva Anupam, Pruthi Praveen K, Sawhney Sujata, Piplani Tarun, Wada Taizo, Yachie Akihiro
Abstract excerpt
Heme oxygenase-1 (HO-1) is a stress-induced enzyme that catalyses the oxidation of heme to biliverdin. The primary deficiency of this enzyme has been shown in HO-1 knockout mice, and is characterized by intrauterine death and chronic inflammation. The first case of human HO-1 deficiency was reported in 1999. Human HO-1 deficiency has been observed to involve the endothelial cells more severely, resulting in...
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