Tumor Suppressor Genes

Question 1

APC

Answer 1

Neg regulator of beta-catenin and WNT path

Colorectal cancer (associated w/ FAP)

Question 2

BRCA1/2

Answer 2

DNA repair protein

Breast, ovarian, pancreatic

Question 3

CDKN2A

Answer 3

p16 (blocks G1 –> S)

melanoma, pancreatic

Question 4

DCC

Answer 4

“deleted in colon cancer”

Question 5

DPC4/SMAD4

Answer 5

“deleted in pancreatic cancer”

Question 6

MEN1

Answer 6

Menin

Question 7

NF1

Answer 7

Neurofibromin (RAS GTPase activating protein)

Neurofibromatosis 1

Question 8

NF2

Answer 8

Merlin (schwannomin protein)

Neurofibromatosis 2

Question 9

PTEN

Answer 9

Tyrosine phosphatase of PIP3

Breast, prostate, endometrial

Question 10

Rb

Answer 10

Inhibits E2F to block G1 –> S

Retinoblastoma, osteosarcoma

Question 11

Tp53

Answer 11

p53 activates p21 to prevent G1 –> S

MOST CANCERS

Li Fraumeni syndrome (mult cancers at early age); breast, sarcoma, leukemia, adrenal gland

Question 12

TSC1

Answer 12

Hamartin protein

tuberous sclerosis

Question 13

TSC2

Answer 13

Tuberin protein

tuberous sclerosis

Question 14

VHL

Answer 14

inhibits hypoxia-inducible factor 1a

VHL disease

Question 15

WT1

Answer 15

transcription factor that regulates urogenital development

Wilms tumor (nephroblastoma)