Updated: 3/11/2020

Tumor Suppressor Genes

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Overview
  • Mechanism
    • loss of function mutation in a tumor suppressor gene
      • regulate cell growth and repair damage
      • see Cell cycle topic
    • because one functioning tumor suppressor gene copy can produce enough functioning protein to prevent neoplasia, two allele alterations needed for neoplasia  
      • two-hit model
      • note many of the inherited cancer disorders have an inherited loss of 1 copy

  • Examples
Gene Associated Tumor Chromosome
APC Colorectal cancer (associated with FAP)
5
p16 Melanoma
9
WT1 Wilms' tumor
11
Rb Retinoblastoma and osteosarcoma 
13
BRCA2 Breast cancer
13
NF1 Neurofibromatosis type 1
17
BRCA1 Breast and ovarian cancer
17
TP53 Most human cancers and Li-Fraumeni syndrome 
17
DPC Pancreatic cancer (stands for deleted in pancreatic cancer)
18
DCC Colon cancer (stands for deleted in colon cancer)
18
NF2 Neurofibromatosis type 2
22

 

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