Human Gene Set: POMEROY_MEDULLOBLASTOMA_DESMOPLASIC_VS_CLASSIC_DN


Standard name POMEROY_MEDULLOBLASTOMA_DESMOPLASIC_VS_CLASSIC_DN
Systematic name M8510
Brief description Top down-regulated marker genes for medulloblastoma classification: desmoplastic vs classic morphology.
Full description or abstract Embryonal tumours of the central nervous system (CNS) represent a heterogeneous group of tumours about which little is known biologically, and whose diagnosis, on the basis of morphologic appearance alone, is controversial. Medulloblastomas, for example, are the most common malignant brain tumour of childhood, but their pathogenesis is unknown, their relationship to other embryonal CNS tumours is debated, and patients' response to therapy is difficult to predict. We approached these problems by developing a classification system based on DNA microarray gene expression data derived from 99 patient samples. Here we demonstrate that medulloblastomas are molecularly distinct from other brain tumours including primitive neuroectodermal tumours (PNETs), atypical teratoid/rhabdoid tumours (AT/RTs) and malignant gliomas. Previously unrecognized evidence supporting the derivation of medulloblastomas from cerebellar granule cells through activation of the Sonic Hedgehog (SHH) pathway was also revealed. We show further that the clinical outcome of children with medulloblastomas is highly predictable on the basis of the gene expression profiles of their tumours at diagnosis.
Collection C2: Curated
      CGP: Chemical and Genetic Perturbations
Source publication Pubmed 11807556   Authors: Pomeroy SL,Tamayo P,Gaasenbeek M,Sturla LM,Angelo M,McLaughlin ME,Kim JY,Goumnerova LC,Black PM,Lau C,Allen JC,Zagzag D,Olson JM,Curran T,Wetmore C,Biegel JA,Poggio T,Mukherjee S,Rifkin R,Califano A,Stolovitzky G,Louis DN,Mesirov JP,Lander ES,Golub TR
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Source species Homo sapiens
Contributed by Jean Junior (MSigDB Team)
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identifier namespace		 AFFY_HuGene
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Version history 3.0: Renamed from POMEROY_DESMOPLASIC_VS_CLASSIC_MD_DN

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