Summary: Tumorbio-6
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1 Tumorbio-6
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What is the effect of Abl in leukemia?
translocation mediated oncogene activation in hematological tumors -
Name the 2 fases and their charact of CML
1) chronic phase = up to >10 years will maintain normal cell acitivity
2) accelerated phase and blastersis (BC-CML) -
What is the effect of Philadelphia chrosomose?
translocation genes c-abl & bcr -> hybrid bcr-abl -
What is the effect of c-bcr?
growth, motility, anti-apoptosis, DNA-repair defect -
What is the effect of fusion proteins
oligomerization resulting in a constitutive activation of PDGF-Rbeta -
What is the effect of class 1 mutations in AML?
activation of a signal transduction pathway (e.g bcr-abl mutations) that stimulate proliferation and survival and has n influence on the differentiation -
What is the effect of class 2 mutations in AML?
changes in transcription factors ( fusion proteins like ETO, point mutations) which block the differntiation -
What are the fases of leukemia?
M0 = minimally differentiated
M1 = myeloblastic leukemia without maturation
M2 = myeloblastic leukemia with maturation
M3 = hypergranular promyelocytic leukemia
M4 = myelomonocytic leukemia
M5 = monocytic leukemia
M6 = erythroleukemia (diguglielmo's disease)
M7 = megakaryoblastic leukemia -
What is the effect of AML FAB (M3)?
promyeolocytes in bone marrow with characteristic granula
hybride receptor blocks differentiation
gets worse through chemo
translocation in PML/RARalfa fusion gene -
What is the effect of RAR/RXR?
binds to dna + recruitment ligand & trnascription repression complex that leads to histone deacetylation -> repression of target gene
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