RNGTT
Overview
RNGTT (RNA Guanylyl Transferase and 5’ Phosphatase) encodes an mRNA capping enzyme involved in co-transcriptional 5’ cap addition. In cancer genomics, RNGTT was identified as a direct transcriptional target of DUX4 in the DUX4/ERG subtype of B-cell ALL: DUX4 binding at the RNGTT locus induces transcription from a non-canonical first exon, analogous to its mechanism at ERG and NSD1. This makes RNGTT a marker of DUX4 transcriptional activity rather than a recurrently mutated driver.
Alterations observed in the corpus
- Transcription from a non-canonical first exon induced by DUX4 binding in DUX4/ERG B-ALL; identified as one of three loci (alongside ERG and NSD1) directly bound by DUX4 (confirmed by ChIP-seq in NALM-6 and Reh cells) among 45 transcripts using non-canonical first exons significantly deregulated in DUX4/ERG ALL PMID:27776115
Cancer types (linked)
- BLL — expression of a non-canonical RNGTT isoform is a feature of the DUX4/ERG ALL subtype (~7.6% of B-ALL; 1,913-patient multi-cohort study); the subtype carries IGH–DUX4 rearrangement and is associated with favorable outcome PMID:27776115
Co-occurrence and mutual exclusivity
- Non-canonical RNGTT transcription co-occurs with DUX4-driven non-canonical expression of ERG (ERGalt isoform) and NSD1 in the same DUX4/ERG ALL subtype PMID:27776115
Therapeutic relevance
- No direct therapeutic relevance described in the corpus; RNGTT is a downstream transcriptional marker of DUX4 activity rather than a target PMID:27776115
Open questions
- Whether non-canonical RNGTT isoform expression contributes functionally to B-ALL pathogenesis or is a bystander effect of DUX4 activity is not addressed in the corpus PMID:27776115
Sources
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