Oncogenes

Question 1

BCR-ABL

Answer 1

oncogene; tyrosine kinase, t(9;22), involved in CML and ALL

Question 2

Bcl-2

Answer 2

oncogene; ch. 18; antiapoptotic molecule: stabilizes mitochondrial membrane and binds/inhibits Apaf-1 (normally induces expression of caspases); involved in follicular [t(14;18)] and undifferentiated lymphomas

Question 3

BRAF

Answer 3

oncogene; serine/threonine kinase; involved in melanoma and non-Hodgkin lymphoma

Question 4

c-kit

Answer 4

oncogene; cytokine receptor; involved in gastrointestinal stromal tumors (GIST); mutation indicates good candidacy for imatinib tx

Question 5

c-myc

Answer 5

oncogene; ch. 8; transcription factor; involved in Burkitt lymphoma [t(8;14)]

Question 6

HER2/neu

Answer 6

oncogene; tyrosine kinase; involved in breast, ovarian, and gastric carcinomas; mutation indicates good candidacy for trastuzumab

Question 7

L-myc

Answer 7

oncogene; transcription factor; involved in lung tumors (small cell)

Question 8

N-myc

Answer 8

oncogene; transcription factor; involved in neuroblastoma

Question 9

RAS

Answer 9

oncogene; GTPase; colon, lung, and pancreatic cancer

Question 10

RET

Answer 10

oncogene; tyrosine kinase; involved in MEN 2A (medullary thyroid cancer, pheochromocytoma, parathyroid hyperplasia) and 2B (medullary thyroid cancer, pheochromocytoma, mucosal neuromas), medullary thyroid cancer

Question 11

APC

Answer 11

tumor suppressor gene; normally down-regulates b-catenin, loss-of-function mutation leads to uncontrolled b-catenin activity/stimulation of growth factors; involved with colorectal cancer and familial adenomatous polyposis (FAP)

Question 12

BRCA1/BRCA2

Answer 12

tumor suppressor genes; DNA repair proteins; involved in breast and ovarian cancer

Question 13

DCC

Answer 13

tumor suppressor gene; “deleted in colon cancer”

Question 14

DPC4/SMAD4

Answer 14

tumor suppressor gene; “deleted in pancreatic cancer”

Question 15

MEN1

Answer 15

tumor suppressor gene; ch. 11; menin; involved in MEN1 (parathyroid adenoma, pituitary adenoma, enteropancreatic tumors)

Question 16

NF1

Answer 16

ch. 17; tumor suppressor gene; neurofibronin, Ras GTPase activating protein; involved in neurofibromatosis type 1

Question 17

NF2

Answer 17

tumor suppressor gene; merlin (schwannomin); involved in neurofibromatosis type 2

Question 18

p16

Answer 18

tumor suppressor gene; cyclin-dependent kinase inhibitor 2A; involved in melanoma

Question 19

p53

Answer 19

tumor suppressor gene; transcription factor for p21, blocks G1 –> S phase by recruiting DNA repair machinery and/or inducing apoptosis via BAX; involved in most human cancers and Li-Fraumeni syndrome

Question 20

PTEN

Answer 20

tumor suppressor gene; involved in breast cancer, prostate cancer, endometrial cancer

Question 21

Rb

Answer 21

tumor suppressor gene; blocks G1 –> S phase; hypophosphorylated protein sequesters E2 and promotes histone deacetylation/methylation, hyperphosphhorylated protein (via Cyclin D/CDK4) releases E2; involved in retinoblastoma and osteosarcoma

Question 22

TSC1

Answer 22

tumor suppressor gene; hamartin protein; involved in tuberous sclerosis

Question 23

TSC2

Answer 23

tumor suppressor gene; tuberin protein; involved in tuberous sclerosis

Question 24

VHL

Answer 24

tumor suppressor gene; inhibits hypoxia inducible factor 1a; involved in von Hippel-Lindau disease (hemangioblastomas, angiomatosis, bilateral renal cell carcinoma, pheochromocytoma), renal cell carcinoma

Question 25

WT1/WT2

Answer 25

ch. 11; tumor suppressor genes; Wilms Tumor (nephroblastoma)