Tumor Suppresor Genes (Gene/Gene Product/Associated Tumor)

Question 1

Tumor suppressor genes

Answer 1

Loss of function leads to increased cancer risk. BOTH alleles must be lost for expression of disease

Question 2

APC

Answer 2

ASSOCIATED TUMOR: Colorectal cancer (associated with FAP)

Question 3

BRCA1/BRCA2

Answer 3

GENE PRODUCT: DNA repair protein

ASSOCIATED TUMOR: Breast and ovarian cancer

Question 4

DCC

Answer 4

GENE PRODUCT: DCC - deleted in colon cancer

ASSOCIATED TUMOR: colon cancer

Question 5

DPC4/SMAD4

Answer 5

GENE PRODUCT: DPC - deleted in pancreatic cancer

ASSOCIATED TUMOR: pancreatic cancer

Question 6

MEN1

Answer 6

GENE PRODUCT: Menin

ASSOCIATED TUMOR: MEN 1

Question 7

NF1

Answer 7

GENE PRODUCT: Ras GTPase activating protein (neurofibromin)

ASSOCIATED TUMOR: Neurofibromatosis type 1

Question 8

NF2

Answer 8

GENE PRODUCT: Merlin (schwannomin) protein

ASSOCIATED TUMOR: Neurofibromatosis type 2

Question 9

p16

Answer 9

GENE PRODUCT: Cycline-dependent kinase inhibitor 2A

ASSOCIATED TUMOR: melanoma

Question 10

p53

Answer 10

GENE PRODUCT: Transcription factor for p21, blocks G1 to S phase
ASSOCIATED TUMOR: most human cancers, Li-Fraumeni syndrome

Question 11

PTEN

Answer 11

ASSOCIATED TUMOR: breast cancer, prostate cancer, endometrial cancer

Question 12

Rb

Answer 12

GENE PRODUCT: Inhibits E2F: blocks G1 to S phase

ASSOCIATED TUMOR: Retinoblastoma, osteosarcoma

Question 13

TSC1

Answer 13

GENE PRODUCT: Hamartin protein

ASSOCIATED TUMOR: tuberous sclerosis

Question 14

TSC2

Answer 14

GENE PRODUCT: Tuberin protein

ASSOCIATED TUMOR: tuberous sclerosis

Question 15

VHL

Answer 15

GENE PRODUCT: Inhibits hypoxia inducible factor 1a

ASSOCIATED TUMOR: von Hippel-Lindau disease, renal cell carcinoma

Question 16

WT1/WT2

Answer 16

ASSOCIATED TUMOR: Wilms Tumor (nephroblastoma)