FREM2 Antibody
Description
Overview of FREM2 Antibody
FREM2 antibodies are used to immunodetect the FREM2 protein, a 351.2 kDa glycoprotein localized to the sublamina densa of basement membranes. Key features include:
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Function: Anchors epithelial layers to underlying tissues, regulates cell adhesion, and supports organ morphogenesis (e.g., skin, eyes, kidneys) .
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Pathological Relevance: Mutations in FREM2 are linked to Fraser syndrome (congenital skin blistering, cryptophthalmos, renal agenesis) and colorectal cancer (CRC) progression .
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Post-Translational Modifications: Includes glycosylation, impacting antibody specificity and binding efficacy .
Applications and Techniques
FREM2 antibodies are employed in diverse experimental workflows:
Fraser Syndrome and Developmental Biology
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FREM2 Knockout Models: Mice lacking Frem2 exhibit bilateral renal agenesis, skin blistering, and neonatal lethality, mimicking Fraser syndrome .
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Antibody Limitations: Commercial antibodies often fail to detect FREM2 in immunogold labeling, highlighting challenges in ECM protein visualization .
Glioblastoma Targeting
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Nanobody Development: A FREM2-specific nanobody (NB3F18) exhibits cytotoxic effects on glioblastoma stem cells (GSCs) via extracellular binding, with minimal impact on normal astrocytes .
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Subcellular Localization: NB3F18 shows differential internalization in GSCs vs. differentiated glioblastoma cells, suggesting cell-type-specific FREM2 dynamics .
Emerging Directions
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Therapeutic Potential: FREM2-targeting nanobodies may enable drug delivery to glioblastoma cells while sparing healthy tissue .
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Diagnostic Biomarkers: FREM2 expression levels in CRC and glioblastoma could serve as prognostic markers, guiding personalized therapies .
Challenges and Future Research
Product Specs
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Target Background
FREM2 is an extracellular matrix protein essential for maintaining the integrity of skin and renal epithelia. It plays a crucial role in epidermal adhesion and is involved in the development of eyelids and the anterior segment of the eye.
- FREM2 is identified as a novel glioblastoma (GB) biomarker and a potential biomarker for glioblastoma stem cells. Surface expression of FREM2 and SPRY1 is observed on GB cells, with SPRY1 showing overexpression in the cytosol of non-malignant astrocytes. PMID: 29734672
- Recessive mutations in FRAS1, FREM2, GRIP1, FREM1, ITGA8, and GREM1, all involved in ureteric bud and metanephric mesenchyme interaction, were identified in 15 of 590 families. PMID: 24700879
- FREM2 gene amplification is associated with mesenchymal differentiation in gliosarcoma. PMID: 22538188
- Heterozygous missense mutations in FREM2 cause non-syndromic congenital abnormalities of the kidney and urinary tract. PMID: 21900877
- A homozygous IVS14 + 1G -- A mutation in FREM2 was identified in two fetuses with Fraser syndrome, suggesting that the syndrome may result from complete or near-complete loss of FREM2 protein function. PMID: 18203166
- A new mutation in FREM2 was identified in families with Fraser syndrome. PMID: 18671281
