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Conservation of Protein Domains Over Different Proteins02:26

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Ribosomal RNA (rRNA) sequence analysis revealed three distinct groups of cells: eukaryotes, bacteria, and archaea. In 1978, Carl R. Woese proposed the concept of domains, a taxonomic level above kingdoms, to differentiate these groups. He suggested that archaea and bacteria, despite their similar appearance, represent separate domains. Domains differ in rRNA, membrane lipid structure, transfer RNA, and antibiotic sensitivity.In this classification, animals, plants, and fungi belong to the...
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Related Experiment Video

Updated: Jan 27, 2026

The Drosophila Imaginal Disc Tumor Model: Visualization and Quantification of Gene Expression and Tumor Invasiveness Using Genetic Mosaics
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FXR1 expression domain in Wilms tumor.

Hannah M Phelps1, Janene M Pierce2, Andrew J Murphy3

  • 1Vanderbilt University School of Medicine, Nashville, TN.

Journal of Pediatric Surgery
|March 22, 2019
PubMed
Summary

Fragile X-Related 1 (FXR1) is consistently expressed in Wilms tumor (WT), particularly in primitive blastema. This RNA-binding protein may label self-renewing kidney progenitor cells.

Keywords:
Embryonal tumorFXR1NephroblastomaPediatric cancerWilms tumor

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Area of Science:

  • Pediatric Oncology
  • Molecular Biology
  • Cancer Research

Background:

  • Wilms tumor (WT) is the predominant childhood kidney cancer globally.
  • Previous proteomic studies identified increased Fragile X-Related 1 (FXR1) expression in Kenyan WT specimens, correlating with poor survival.
  • FXR1, an RNA-binding protein, is linked to adverse outcomes in adult cancers.

Purpose of the Study:

  • To validate and characterize the expression patterns of FXR1 within Wilms tumor.
  • To investigate the cellular and subcellular localization of FXR1 in WT and kidney tissues.
  • To explore the broader expression and potential targets of FXR1 in pediatric malignancies.

Main Methods:

  • Quantitative gene expression analysis of FXR1 in WT, adjacent, adult, and fetal kidney tissues.
  • Immunoperoxidase staining to determine the cellular and subcellular distribution of FXR1.
  • RNA-sequencing of FXR1 in WT and other pediatric tumors to assess its expression landscape.

Main Results:

  • FXR1 was universally detected in 82 WT specimens, irrespective of clinical or histological factors.
  • Cytosolic FXR1 staining was most intense in blastemal cells, with variable expression in epithelial and stromal components.
  • FXR1 expression in WT was significantly higher than in adult/adjacent kidney but comparable to fetal kidney.
  • RNA-sequencing confirmed FXR1 expression in various pediatric tumors, notably rhabdomyosarcoma and WT.

Conclusions:

  • FXR1 demonstrates consistent expression across a wide range of WT samples, with peak expression in the primitive blastema.
  • FXR1 expression in WT mirrors that of fetal kidney, suggesting a role in early development or progenitor cells.
  • FXR1 may identify a specific self-renewing progenitor cell population within the fetal kidney.