Professor Chris Jones, Glioma team
Paediatric high grade and diffuse intrinsic pontine glioma
The Jones laboratory is primarily focused on high grade gliomas which arise in children, either supratentorially, or within the brainstem. These tumours appear to have key biological and clinical distinctions, both to each other, and histologically similar lesions in adults.
To this end we have current projects focussed on:
- Histone H3.3 G34R/V mutations in cerebral hemispheric tumours of children and young adults, and the mechanisms by which they maintain a stem/progenitor cell phenotype and upregulate MYCN in the developing cortex.
- ACVR1 mutations in DIPG, and the link to identical germline variants in fibrodysplasia ossificans progressiva, an autosomal dominant syndrome in which aberrant cellular differentiation drives progressive heterotopic ossification.
- Identification of novel drivers of rare CNS entities in children and young adults, including congenital glioblastoma, astroblastoma and gliomatosis cerebri, and the construction of a molecular taxonomy of glial tumours across all ages.
In addition, we wish to more fully characterise and functionalise the intratumoral heterogeneity with glioblastoma, with a particular focus on the likely distinct evolutionary dynamics of the childhood disease.