CELL: The double mutations of histones and PDGFRA promote the occurrence of glioma

Advanced gliomas (HGGs) are deadly primary brain tumors and are the main cause of death in children and adolescents.
these tumors often carry physical mutations or ostogenesic modifications of genes that encode histogene 3 (H3) mutations, with significant neuroanatomy and age specificity.
HGGs in adolescents and young adults (12-35 years old) occur mainly in the hemisphere of the brain, and large amounts of HGGs are thought to be an ostogenesic genetic disease.
more than 30% of these HGGs have hybrid mutations with non-standard H3.3 variants, resulting in the replacement of amino acids in glycine 34 transaminine or proline (G34R/V).
G34R/V tumor research is inadequate and is likely to be underestimated because of its unique heterogeneity of histopathology and the varying degrees of neuron-glial dual-region presence, leading to misdiagnosis.
fact, nearly 30% of the central nervous system's original neuro-exosteeric tumors (CNS-PNETs), now obsolete hybrid high-grade neuron tumor entities, have been shown to be G34R/V mutant HGGs.
On the other hand, at the molecular level, G34R/V HGGs exhibit uniform characteristics; they carry almost invantly mutations in α thalassemia/mentally retarded syndrome X-linked (ATRX) and tumor protein p53 (TP53), lack the immune response of the glioblast marker OLEG2, and differ from other glioma entities based on DNA methylation.
recently, researchers published in the journal CELL showed that 50% of G34R/V tumors (n s 95) have activated PDGFRA mutations that show strong selection pressure when they relapse.
is thought to be a glioma, G34R/V tumors actually occur in the intermediate neuron progenitocytes expressed by GSX2/DLX, and the G34R/V mutation impairs neuron differentiation.
of PDGFRA may promote the common selection of PDGFRA by connecting the chromatin ring of PDGFRA with the GSX2 regulatory element, and promote PDGFRA overextending and mutation.
at the single-cell level, G34R/V tumor has the dual identity of neuron/star glial, lacks the characteristics of less protrusion glial, and is actively inhibited by GSX2/DLX-mediated cell fate norms.
G34R/V may be available in tumor maintenance, and the mutant PDGFRA is powerfully carcinogenic.
, the results open up a new avenue for research into deadly tumors.
G34R/V glioma is a neuron malignant tumor in which the intermediate neuron prelude cells are differentiated and stagnated by the G34R/V mutation, and the occurrence of malignant glial is facilitated by the joint selection of a potentially targeted pathway PDGFRA signal.
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