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Target intelligence / Profile preview
FRAS1-related extracellular matrix protein 2 (FREM2)
- Target
- FREM2
- Molecular classification
- Extracellular matrix protein, Calcium-binding protein, Structural protein, Other
01
Overview
FRAS1-related extracellular matrix protein 2 (FREM2) is an extracellular matrix protein essential for tissue morphogenesis and the structural integrity of various epithelial and mesenchymal tissues, particularly during embryonic development[1][4][5]. FREM2 is a calcium-binding protein and a structural component of the FRAS/FREM complex, which anchors the basement membrane and supports tissue cohesion[4]. Mutations in the FREM2 gene are a known cause of Fraser syndrome, leading to defects such as cryptophthalmos, syndactyly, and kidney abnormalities[4]. FREM2 is also implicated in neural tube and vascular development[1][5]. Recent studies indicate that the activation level of the FREM2 pathway serves as a robust diagnostic and prognostic biomarker in gliomas, including glioblastoma, where higher pathway activity correlates with disease aggressiveness and poor prognosis[2]. FREM2 is not classically considered a direct therapeutic target, and no drugs are known to directly interact with it.
- Other names
- FRAS1 related extracellular matrix 2DKFZp686J0811ECM3 homologCRYPTOPFRASRS2FRAS1-related extracellular matrix protein 2FRAS1 related extracellular matrix protein 2
02
Biological functions
Tissue morphogenesisIntegrity of the skin epitheliumBasement membrane adhesionRegulation of extracellular matrix organizationVascular stability and developmentKidney and organ development
03
Disease associations
Congenital malformation syndrome (Fraser syndrome)Neural tube defectsRenal developmental abnormalitiesCancer (notably glioblastoma and low-grade gliomas)Other developmental disorders
04
Safety considerations
Mutations can cause severe congenital malformations including Fraser syndrome (cryptophthalmos, syndactyly, kidney malformations)Loss of protein function leads to fragile basement membranes and extensive developmental abnormalities
05
Biomarkers
FREM2 molecular pathway activation is a prognostic and diagnostic biomarker for glioma subtypes (e.g., glioblastoma, low-grade glioma)
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