Anti-Hemojuvelin antibody [C1]

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

Hemojuvelin also known as HJV or RGMc is a glycosylphosphatidylinositol-anchored protein that plays a significant role in iron homeostasis. It has a molecular mass of about 42 kDa. Expression of hemojuvelin is mainly observed in the liver heart and skeletal muscles. This protein is essential for maintaining systemic iron levels and regulates how much iron is absorbed and stored in the body.

Biological function summary

Hemojuvelin interacts with other proteins to modulate iron metabolism. It acts as a coreceptor for bone morphogenetic proteins (BMPs) enhancing BMP signaling which is critical in regulating the peptide hormone hepcidin. Hemojuvelin itself is not part of a larger complex but it works closely with BMPs to control hepcidin expression. Hepcidin is the key hormone that dictates iron absorption and its release from macrophages maintaining iron balance.

Pathways

Hemojuvelin functions primarily within the BMP signaling and hepcidin regulatory pathways. The interaction with BMP6 is particularly critical. The BMP6-hemojuvelin complex boosts the SMAD signaling cascade resulting in increased hepcidin expression. This cascade impacts iron levels in the body by limiting intestinal iron absorption and controlling iron recycling by macrophages.

Associated diseases and disorders

Hemojuvelin mutations can lead to juvenile hemochromatosis a condition characterized by excessive iron buildup in the body. This protein's dysfunction can severely affect iron homeostasis leading to detrimental health outcomes. Moreover its connection to the regulation of hepcidin links hemojuvelin to conditions like anemia of chronic disease where hepcidin expression is aberrantly increased or decreased. Understanding the interaction of hemojuvelin and hepcidin provides insights into the mechanisms behind these iron-related disorders.