# The Molecular function of ecto 5' nucleotidase in fusion-negative rhabdomyosarcoma

> **NIH NIH F31** · UT SOUTHWESTERN MEDICAL CENTER · 2024 · $17,276

## Abstract

Project Summary/Abstract
 Rhabdomyosarcoma (RMS) is an aggressive pediatric soft tissue sarcoma characterized by the lack of
myogenic differentiation. RMS is divided in two molecular categories: fusion positive (FP-RMS) carrying a
balanced chromosomal translocation generating an oncogenic Pax3-FOXO1 or Pax7-FOXO1 protein, and fusion
negative (FN-RMS) that does not include the fusion event. Current treatments for FN-RMS remain poor and the
long-term event-free survival rate for FN-RMS metastatic patients is below 30%. TWIST2 transcription factor
represses myogenesis by inhibiting MyoD function and knockdown of TWIST2 in FN-RMS is shown to inhibit
tumor cell growth and enhance myogenic differentiation. To understand the mechanisms underlying TWIST2
function in FN-RMS, we sought to identify its direct transcriptional targets. Among them, we identified NT5E,
encoding an ecto 5’-nucleotidase, as a direct target gene of TWIST2. NT5E’s main role is to convert adenosine-
monophosphate to adenosine in the purinergic signaling pathway. NT5E has been shown to promote cancer
progression, invasion and metastasis in human melanoma and breast cancer. However, it’s possible role in FN-
RMS has not been explored. In this proposal, we hypothesize that NT5E contributes to FN-RMS tumor formation
and progression through the activation of the purinergic signaling pathway. To test this hypothesis, we will
investigate the contribution of NT5E expression to the pathogenesis of FN-RMS in vitro and in vivo. Then, we
will delineate the molecular mechanism whereby NT5E promotes FN-RMS. Completion of this research plan will
push the boundaries of our knowledge regarding the role of NT5E in FN-RMS pathogenesis with the ultimate
goal of developing novel therapeutic strategies for FN-RMS.

## Key facts

- **NIH application ID:** 10833007
- **Project number:** 5F31CA271675-03
- **Recipient organization:** UT SOUTHWESTERN MEDICAL CENTER
- **Principal Investigator:** Karla Gabriela Cano Hernandez
- **Activity code:** F31 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2024
- **Award amount:** $17,276
- **Award type:** 5
- **Project period:** 2022-05-01 → 2024-09-02

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10833007

## Citation

> US National Institutes of Health, RePORTER application 10833007, The Molecular function of ecto 5' nucleotidase in fusion-negative rhabdomyosarcoma (5F31CA271675-03). Retrieved via AI Analytics 2026-05-24 from https://api.ai-analytics.org/grant/nih/10833007. Licensed CC0.

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