Monkey Crew Against D.I.P.G.   Childhood Brainstem Cancer Awareness                     
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  What is D.I.P.G?

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Diffuse Intrinsic Pontine Glioma is an aggressive, inoperable brain-stem tumour that grows throughout the supportive tissues of the pons.
Pontine: The Pons (3) connect the upper (1) and lower (2) parts of the brain; part of the brain-stem between the medulla oblongata (4) and the midbrain  
Diffuse: Spread throughout the connective tissue; not localized or defined; not a solid mass Intrinsic: Originating and wholly within the pons
Glioma: tumours that form in the glial or supportive neurological tissue of the brain
The World Health Organization, as of 2016, has started using the term Diffuse midline glioma H3 K27M-mutant (considered a WHO grade IV tumour) to describe tumours with the H3 K27M mutation which includes 80% of diffuse intrinsic pontine gliomas. However, this term does not cover the 20% of D.I.P.G. tumours without this mutation. The new classification also covers tumours in areas other than the pons, notably the thalamus where a significant number of pediatric gliomas also harbor the H3K27M mutation but these non-pontine diffuse gliomas, despite sharing the histone mutation, differ from D.I.P.G. in that we do not see the same partner mutations in the other tumors that we see in D.I.P.G. Therefore, diffuse midline glioma and D.I.P.G. should NOT be used interchangeably (even though they have been). -Information from Cynthia Hawkins, Michael Mosier Defeat DIPG, & DIPG Research  

What Causes D.I.P.G.?

Little is known about the causes of D.I.P.G. and researchers have only had access to D.I.P.G. cells for study in the last 6 years. In this time, they have made a few important discoveries. Researchers are looking at the biology of D.I.P.G. tumours for such problems as: abnormal Gene expression too much or too little Methylation Genetic mutations Structural changes in the genome   Observations have led researchers to categorize DIPG into three very different molecular subgroups: (these are very basic descriptions of the subtypes to give you an idea of differences) 1) MYCN high level of methylation no Histone mutations multiple rearrangements on chromosome 2 high amplification of MYCN and ID2 genes 2) Silent stable genomes compared to MYCN and H3-K27M groups few mutations, although some mutations in Histone H3, TP53 and ACVR1 genes 3) H3 - K27M largest subgroup highly unstable genomes highly mutated Histone genes (H3.3 or H3.1) some TP53 and ACVR1 gene mutations low level of methylation What are Histones? DNA wraps around histones, which are special chromosomal proteins This wrapping compacts DNA so that the long DNA strands can fit inside the nuclei of the cell histones can play a role in whether genes are turned "on" or "off" What happens in these D.I.P.G. Histone mutations? one particular amino acid Lysine, is exchanged for another one, Methionine this leads to less expression of PRC2 PRC2 usually turns off some gene expression through histone methylation when PRC2 is suppressed, genes which should be "off" are more accessible and are turned "on" 80% of DIPG patients have mutations in Histone genes H3.3 or H3.1. Whereas,  studies of other cancers reveal genetic mutations in only 5% of cases.
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Monkey Crew Against D.I.P.G. Childhood Brainstem Cancer Awareness
               © All rights reserved Monkey Crew Against D.I.P.G. 2016
Diffuse: Spread throughout the connective tissue; not localized or defined; not a solid mass    Intrinsic: Originating and wholly within the pons        Pontine: The Pons (3) connect the upper (1) and lower (2) parts of the brain; part of the brainstem between the medulla oblongata (4) and the midbrain   Glioma: tumours that form in the glial or supportive neurological tissue of the brain  Diffuse midline glioma H3 K27M–mutant
What is D.I.P.G.? Diffuse Intrinsic Pontine Glioma is an aggressive, inoperable brainstem tumour that grows throughout the supportive tissues of the pons.
The World Health Organization, as of 2016, has started using the term Diffuse midline glioma H3 K27M-mutant (considered a WHO grade IV tumour) to describe tumours with the H3 K27M mutation which includes 80% of diffuse intrinsic pontine gliomas. However, this term does not cover the 20% of D.I.P.G. tumours without this mutation. The new classification also covers tumours in areas other than the pons, notably the thalamus where a significant number of pediatric gliomas also harbor the H3K27M mutation but these non-pontine diffuse gliomas, despite sharing the histone mutation, differ from D.I.P.G. in that we do not see the same partner mutations in the other tumors that we see in D.I.P.G. Therefore, diffuse midline glioma and D.I.P.G. should NOT  be used interchangeably (even though they have been). -Information from Cynthia Hawkins, Michael Mosier Defeat DIPG, & DIPG Research

What Causes D.I.P.G.?

Little is known about the causes of D.I.P.G. and researchers have only had access to D.I.P.G. cells for study in the last 6 years. In this time, they have made a few important discoveries. Researchers are looking at the biology of D.I.P.G. tumours for such problems as: abnormal Gene expression too much or too little Methylation Genetic mutations Structural changes in the genome   Observations have led researchers to categorize DIPG into three very different molecular subgroups: (these are very basic descriptions of the subtypes to give you an idea of differences) 1) MYCN high level of methylation no Histone mutations multiple rearrangements on chromosome 2 high amplification of MYCN and ID2 genes 2) Silent stable genomes compared to MYCN and H3- K27M groups few mutations, although some mutations in Histone H3, TP53 and ACVR1 genes 3) H3 - K27M largest subgroup highly unstable genomes highly mutated Histone genes (H3.3 or H3.1) some TP53 and ACVR1 gene mutations low level of methylation What are Histones? DNA wraps around histones, which are special chromosomal proteins This wrapping compacts DNA so that the long DNA strands can fit inside the nuclei of the cell histones can play a role in whether genes are turned "on" or "off" What happens in these D.I.P.G. Histone mutations? one particular amino acid Lysine, is exchanged for another one, Methionine this leads to less expression of PRC2 PRC2 usually turns off some gene expression through histone methylation when PRC2 is suppressed, genes which should be "off" are more accessible and are turned "on" 80% of DIPG patients have mutations in Histone genes H3.3 or H3.1. Whereas,  studies of other cancers reveal genetic mutations in only 5% of cases.
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