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EZR

6q25.3 HGNC:12691 Entrez:7430 MIM.123900

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Defintion: EZR (VIL2 or ezrin or cytovillin) is a microvillar cytoplasmic peripheral membrane protein that is expressed strongly in placental syncytiotrophoblasts and in certain human tumors.

EZR (ezrin) is a component of the microvilli of intestinal epithelial cells that serves as a major cytoplasmic substrate for certain protein-tyrosine kinases.

ERM proteins (Ezrin-Radixin-Moesin proteins)

Ezrin (VIL2), radixin (RDX) (MIM.179410), and moesin (MSN) (MIM.309845), the so-called ERM proteins (Ezrin-Radixin-Moesin proteins), act as linkers between the plasma membrane and the actin cytoskeleton.

ERM proteins are involved in a variety of cellular functions, such as cell adhesion, cell migration, and the organization of cell surface structures. ERM proteins are highly homologous, both in protein structure and in functional activity, with merlin/schwannomin, the NF2 tumor suppressor protein (MIM.607379).

Pathology

- metastasis of osteosarcoma and rhabdomyosarcoma

  • signaling between metastasis-associated cell-surface molecules and signal transduction components

- metastasis of osteosarcoma

  • The ezrin expression is associated with more aggressive osteosarcomas and with metastasis.
  • The ezrin expression is associated with the development of metastasis and poor outcome in osteosarcoma.
  • Ezrin is present in the cytoplasm and after phosphorylation assumes an active form and links F-actin to the cell membrane.
  • Phosphorylated ezrin is located in the nucleus of the osteosarcoma cell. (#20348881#)
  • The phosphorylated ezrin Tyr354 nuclear localization suggests its possible role as a nuclear factor in osteosarcoma. (#20348881#)
  • The phosphorylated ezrin Thr567 phosphorylation may not be necessary in osteosarcoma metastatic progression but it was modulated. (#20348881#)

Immunostaining

- Ezrin is a useful tool for differential diagnosis between chondroblastic osteosarcoma and chondrosarcoma. (#19050913#)

See also

- VLNs, ITGs, VCL
- ERM proteins

References

- Phosphorylated ezrin is located in the nucleus of the osteosarcoma cell. Di Cristofano C, Leopizzi M, Miraglia A, Sardella B, Moretti V, Ferrara A, Petrozza V, Della Rocca C. Mod Pathol. 2010 Jul;23(7):1012-20. PMID: #20348881# [Free]

- Ezrin immunohistochemical expression in cartilaginous tumours: a useful tool for differential diagnosis between chondroblastic osteosarcoma and chondrosarcoma. Salas S, de Pinieux G, Gomez-Brouchet A, Larrousserie F, Leroy X, Aubert S, Decouvelaere AV, Giorgi R, Fernandez C, Bouvier C. Virchows Arch. 2009 Jan;454(1):81-7. PMID: #19050913#

- Hunter KW. Ezrin, a key component in tumor metastasis. Trends Mol Med. 2004 May 1;10(5):201-204. PMID: #15121044#