TMEM106B
Overview
TMEM106B (Transmembrane Protein 106B) encodes a lysosomal transmembrane protein associated with frontotemporal lobar degeneration when polymorphic. As a fusion partner, TMEM106B contributes regulatory elements (promoter/enhancer) that drive overexpression of the kinase partner. The TMEM106B-BRAF fusion has been identified in pleomorphic xanthoastrocytoma (PXA) and other low-grade gliomas, creating a constitutively active BRAF kinase.
Alterations observed in the corpus
- TMEM106B-BRAF fusion identified in a pediatric pleomorphic xanthoastrocytoma (PXA) in the PIPseq pediatric precision sequencing program (n=101, Columbia University); designated as a MEK-inhibitor target. PMID:28007021
Cancer types (linked)
- PXA: TMEM106B-BRAF fusion creates a constitutively active BRAF kinase; MEK inhibitors (trametinib, cobimetinib) and BRAF inhibitors are the relevant targeted class. PMID:28007021
Co-occurrence and mutual exclusivity
- No additional co-mutation data reported in the corpus for this fusion case.
Therapeutic relevance
- TMEM106B-BRAF fusion is a MEK-inhibitor target designated in the PIPseq cohort; standard approaches include BRAF-V600-directed inhibitors or MEK inhibitors depending on fusion-specific BRAF activity. PMID:28007021
Open questions
- Optimal targeted therapy (BRAF vs. MEK inhibitor) for non-V600E BRAF fusions in PXA requires further clinical data. PMID:28007021
Sources
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