biological source
rabbit
conjugate
unconjugated
antibody form
culture supernatant
antibody product type
primary antibodies
clone
MRQ-37, monoclonal
description
For In Vitro Diagnostic Use in Select Regions (See Chart)
form
buffered aqueous solution
species reactivity
human
packaging
vial of 0.1 mL concentrate (354R-14)
vial of 0.5 mL concentrate (354R-15)
bottle of 1.0 mL predilute (354R-17)
vial of 1.0 mL concentrate (354R-16)
bottle of 7.0 mL predilute (354R-18)
manufacturer/tradename
Cell Marque®
technique(s)
immunohistochemistry (formalin-fixed, paraffin-embedded sections): 1:50-1:200
isotype
IgG
control
ASPS, melanoma
shipped in
wet ice
storage temp.
2-8°C
visualization
nuclear
Gene Information
human ... TFE3(7030)
Analysis Note
![]() IVD | ![]() IVD | ![]() IVD | ![]() RUO |
General description
Xp11 translocation renal cell carcinomas (RCC) are a recently recognized subset of RCC, characterized by chromosome translocations involving the Xp11.2 break point and resulting in gene fusions involving the TFE3 transcription factor gene that maps to this locus. Xp11 translocation RCC represents the most common type of RCC in children, but is less frequent on a percentage basis in adults. Morphologically, these neoplasms frequently show papillary architecture and clear cytoplasm, and frequently have associated psammoma bodies. Immunohistochemically, these neoplasms under-expresses epithelial markers such as anti-cytokeratin and anti-epithelial membrane antigen (anti-EMA) compared with typical adult type RCC. The most sensitive and specific immunohistochemical marker for the Xp11 translocation RCC is nuclear labeling of TFE3 protein, which reflects over-expression of the resulting fusion proteins relative to native TFE3. Alveolar soft part sarcoma (ASPS) is an uncommon soft tissue sarcoma of undertain differentiation. It presents in younger patients, often in the extremities. Despite relatively high rates of metastasis, patients often experience prolonged survival in the metastatic setting relative to others. The hallmark of ASPS is a chromosomal rearrangement at 17q25 and Xp11.2 engendering an ASPSCR1-TFE3 fusion gene responsible for an aberrant transcription factor presumably enabling pathogenesis.This aberrant chimeric transcription factor retains the N-terminal DNA binding domain encoded by TFE3 while the ASPSCR1 encoded portion probably provides domain(s) modulating gene expression. The presence of this ′super-activated′ transcription factor may induce the expression of numerous molecules contributing to ASPS diagnosis, progression, and metastasis.
Other Notes
For Technical Service please contact: 800-665-7284 or email: service@cellmarque.com
TFE3 Positive Control Slides, Product No. 354S, are available for immunohistochemistry (formalin-fixed, paraffin-embedded sections).
Physical form
Solution in Tris Buffer, pH 7.3-7.7, with 1% BSA and <0.1% Sodium Azide
Preparation Note
Download the IFU specific to your product lot and formatNote: This requires a keycode which can be found on your packaging or product label.
Legal Information
Cell Marque is a registered trademark of Merck KGaA, Darmstadt, Germany
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此项目有
Pedram Argani et al.
Clinics in laboratory medicine, 25(2), 363-378 (2005-04-26)
A significant proportion of RCCs of children and young adults bear specific chromosome translocations that result in gene fusions that involve members of the MiTF/TFE transcription factor family. These include the Xp11-translocation carcinomas, which bear TFE3 gene fusions, and the
Alexander J Lazar et al.
Histopathology, 55(6), 750-755 (2009-12-17)
The molecular signature of alveolar soft part sarcoma (ASPS) is a specific der(17)t(X;17)(p11.2;q25) translocation, resulting in a chimeric transcription factor (ASPSCR1-TFE3). When this disease is no longer amenable to surgical curative intervention, uniformly efficacious therapies are lacking. The aim of
The evolving story of renal translocation carcinomas.
Pedram Argani et al.
American journal of clinical pathology, 126(3), 332-334 (2006-08-02)
Pedram Argani et al.
The American journal of surgical pathology, 27(6), 750-761 (2003-05-27)
We report the aberrantly strong nuclear immunoreactivity for the C-terminal portion of TFE3 protein in tumors characterized by chromosome translocations involving the TFE3 gene at Xp11.2. This group of tumors includes alveolar soft part sarcoma and a specific subset of
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