PRRX1
Overview
PRRX1 (Paired Related Homeobox 1) is a transcription factor involved in mesenchymal cell identity, epithelial-mesenchymal transition (EMT), and limb/craniofacial development. In neuroblastoma, PRRX1 is a defining mesenchymal marker expressed in both the undifferentiated nC3 and the mesenchymal stromal (MSC) nC1 tumor clusters of high-risk disease.
Alterations observed in the corpus
- PRRX1 is significantly expressed in both the undifferentiated nC3 and MSC nC1 clusters of high-risk neuroblastoma tumors in a single-nuclei RNA-seq study of 11 tumors (Smart-Seq2, 3,212 nuclei). RNAscope in situ validation confirmed absence of PRRX1 expression in a low-risk stage 4S tumor (K6). PMID:34493726
Cancer types (linked)
- NBL — mesenchymal marker of undifferentiated nC3 and stromal MSC nC1 clusters in high-risk neuroblastoma; confirmed absent in low-risk stage 4S by RNAscope. High-risk tumors have significantly higher proportions of mesenchymal cells (FDR <0.01, chi-square). PMID:34493726
Co-occurrence and mutual exclusivity
- Co-expressed with YAP1 and PDGFRA in undifferentiated/mesenchymal clusters; PDGFRB is more specifically confined to MSC nC1. The mesenchymal program (PRRX1/YAP1/PDGFRA) is a shared feature of nC3 and nC1 in high-risk neuroblastoma. PMID:34493726
Therapeutic relevance
- No direct targeted therapy is reported in the corpus for PRRX1 in neuroblastoma.
Open questions
- Whether PRRX1 drives mesenchymal identity in neuroblastoma or merely marks it, and whether targeting this program is feasible, are unresolved. PMID:34493726
Sources
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