Hemojuvelin and bone morphogenetic protein (BMP) signaling in iron homeostasis

• Main Authors: Amanda B Core (Author), Susanna eCanali (Author), Jodie L. Babitt (Author)
• Format: Book
• Published: Frontiers Media S.A., 2014-05-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 1663-9812; 10.3389/fphar.2014.00104
• Keywords: Hemochromatosis; Iron; hepcidin; bone morphogenetic protein; Hemojuvelin; repulsive guidance molecule; Therapeutics. Pharmacology; RM1-950
• Source: Frontiers in Pharmacology, Vol 5 (2014)

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.