Physiological importance of heme oxygenase-1 on hemopoiesis, inflammation and immunology system

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Heme oxygenase-1(HO-1) is the rate-limiting enzyme that adds an oxygen molecule to the porphyrin ring of heme, thereby catalyzing to biliverdin, free iron (finally to bilirubin and ferritin, respectively) and carbon monoxide. In 1999, the first case of human HO-1 deficiency was reported by Yachie et al. at our Department of Pediatrics, Kanazawa University Hospital The male patient suffered from generalized severe inflammation since the age of 2, and died at 6 years of age. Quite recently the 2nd case was discovered and reported. Aggressive clinical and patho-physiological studies about these two cases and some experimental investigations have shown detailed clinical findings of HO-1 deficiency and revealed that the HO-1 enzyme is crucial for development and growth of human beings on hematopoiesis, immunological system and the defense mechanism against inflammation and oxidative stresses.