Posttranslational stability of the heme biosynthetic enzyme ferrochelatase is dependent on iron availability and intact iron-sulfur cluster assembly machinery

Blood, 28 January 2010, Vol. 115, No. 4, pp. 860-869.

Daniel R. Crooks1,2, Manik C. Ghosh2, Ronald G. Haller3, Wing-Hang Tong2, and Tracey A. Rouault2


1 Department of Biochemistry, Molecular and Cellular Biology, Georgetown University Medical Center, Washington, DC; 2 Molecular Medicine Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD; and 3 Department of Neurology, University of Texas Southwestern Medical Center and Veterans Administration North Texas Medical Center, and Neuromuscular Center, Institute for Exercise and Environmental Medicine, Dallas

Keywords:  ferrochelatase, iron-sulfur [2Fe-2S] cluster, posttranscriptional regulation of ferrochelatase,  in vivo.  We propose that decreased heme biosynthesis resulting from impaired Fe-S cluster assembly can contribute to the pathogenesis of diseases caused by defective Fe-S cluster biogenesis.