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glioma susceptibility 1 (GLM1)

Gliomas are central nervous system neoplasms derived from glial cells and comprise astrocytomas, glioblastoma multiforme, oligodendrogliomas, and ependymomas (131445).

Gliomas are known to occur in association with several well-defined hereditary tumor syndromes such as mismatch repair cancer syndrome (276300), melanoma-astrocytoma syndrome (155755), neurofibromatosis-1 (NF1; 162200) and NF2 (101000), tuberous sclerosis (see 191100), Li-Fraumeni syndrome (151623), and Turcot syndrome (276300). Familial clustering of gliomas often occurs in the absence of these tumor syndromes, however.

Methylation of the MGMT promoter is related to the responsiveness of gliomas (137800) to alkylating agents (drugs). Knowledge of methylation of the MGMT promoter in gliomas is a useful predictor of the responsiveness of the tumors to alkylating agents.


Related patway(s): direct reversal (DR)

DNAtraffic protein(s) related to disease: MGMT

OMIM: 137800

Last modification date: July 30, 2011