Mutations in hemojuvelin (HJV) are the most common cause of the juvenile-onset form of the iron overload disorder hereditary hemochromatosis. The discovery that HJV functions as a co-receptor for the bone morphogenetic protein (BMP) family of signaling molecules helped to identify this signaling pathway as a central regulator of the key iron hormone hepcidin in the control of systemic iron homeostasis. This review highlights recent work uncovering the mechanism of action of HJV and the BMP-SMAD signaling pathway in regulating hepcidin expression in the liver, as well as additional studies investigating possible extra-hepatic functions of HJV. This review also explores the interaction between HJV, the BMP-SMAD signaling pathway and other regulators of hepcidin expression in systemic iron balance. © 2014 Core, Canali and Babitt.
CITATION STYLE
Core, A. B., Canali, S., & Babitt, J. L. (2014). Hemojuvelin and bone morphogenetic protein (BMP) signaling in iron homeostasis. Frontiers in Pharmacology. Frontiers Research Foundation. https://doi.org/10.3389/fphar.2014.00104
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