Glypican 3

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Glypican-3

Schematic of the glypican-3 protein

Glypican-3 (GPC3) is a member of the glypican family of heparan sulfate proteoglycans that are attached to the cell surface via a glycosylphosphatidylinositol (GPI) anchor. Glypicans are involved in the regulation of cell growth and differentiation, and GPC3, in particular, has been implicated in various biological processes and diseases.

Structure

Glypican-3 is composed of a core protein to which heparan sulfate chains are attached. The protein is anchored to the cell membrane by a GPI anchor. The schematic representation of the glypican-3 protein shows its modular structure, including the N-terminal and C-terminal domains, which are important for its function.

Function

GPC3 plays a crucial role in modulating the activity of growth factors such as Wnt, Hedgehog, and FGF. It acts as a co-receptor, facilitating the interaction of these growth factors with their respective receptors on the cell surface. This modulation is essential for normal embryonic development and tissue homeostasis.

Clinical Significance

Role in Cancer

Glypican-3 is overexpressed in several types of cancer, most notably hepatocellular carcinoma (HCC). It is considered a potential biomarker for the diagnosis and prognosis of HCC. The expression of GPC3 in tumors can be targeted for therapeutic purposes, including the development of GPC3-specific antibodies and CAR T-cell therapies.

Simpson-Golabi-Behmel Syndrome

Mutations in the GPC3 gene are associated with Simpson-Golabi-Behmel syndrome, a rare genetic disorder characterized by pre- and postnatal overgrowth, facial dysmorphism, and an increased risk of developing embryonal tumors. This syndrome highlights the importance of GPC3 in growth regulation.

Research Directions

Ongoing research is focused on understanding the precise molecular mechanisms by which GPC3 regulates growth factor signaling. Additionally, there is significant interest in developing GPC3-targeted therapies for cancer treatment, as well as exploring its role in other diseases.

Related Pages

Template:Glypicans

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Contributors: Prab R. Tumpati, MD