HOTAIR
Overview
HOTAIR is a long non-coding RNA encoded within the HOXC cluster on chromosome 12q13. It functions as an epigenetic regulator, recruiting the PRC2 complex to silence target genes. In cancer, HOTAIR is frequently over-expressed and has been linked to aggressive phenotypes and poor prognosis in multiple tumor types. In malignant rhabdoid tumors (MRT), HOTAIR is covered by an MRT-specific super-enhancer within the HOXC cluster, leading to marked over-expression relative to normal tissues.
Alterations observed in the corpus
- HOTAIR is covered by an MRT-specific super-enhancer in the HOXC cluster and is over-expressed in extra-cranial MRT compared to normal tissues, paralleling observations in AT/RT; this super-enhancer gain occurs in the context of SMARCB1 biallelic inactivation and global loss of H3K27me3. PMID:26977886
Cancer types (linked)
- MRT (Malignant Rhabdoid Tumor): HOTAIR over-expression is a feature of the MRT epigenomic landscape driven by HOX-cluster super-enhancer acquisition following SMARCB1 loss. PMID:26977886
Co-occurrence and mutual exclusivity
- HOTAIR over-expression co-occurs with SMARCB1 biallelic inactivation and gain of MRT-specific super-enhancers at HOX clusters in extra-cranial MRT. PMID:26977886
Therapeutic relevance
- The HOX-cluster super-enhancer landscape, including HOTAIR, is highlighted as a candidate epigenetic dependency in MRT; no specific targeting agents are tested in this study. PMID:26977886
Open questions
- Whether HOTAIR over-expression is mechanistically required for MRT maintenance or is an epiphenomenon of SMARCB1 loss and consequent chromatin remodeling remains untested. PMID:26977886
Sources
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