Pancytopenia and thrombosis defects in zebrafish mutants of Fanconi anemia genes
- Raman, R., Ramanagoudr-Bhojappa, R., Dhinoja, S., Ramaswami, M., Carrington, B., Jagadeeswaran, P., Chandrasekharappa, S.C.
- Blood cells, molecules & diseases 93: 102640 (Journal)
- Registered Authors
- Chandrasekharappa, Settara, Jagadeeswaran, Pudur
- Fanconi anemia, Pancytopenia, Thrombocytopenia, Zebrafish knockouts
- MeSH Terms
- DNA Damage
- Fanconi Anemia*/genetics
- 34991062 Full text @ Blood Cells Mol. Dis.
Raman, R., Ramanagoudr-Bhojappa, R., Dhinoja, S., Ramaswami, M., Carrington, B., Jagadeeswaran, P., Chandrasekharappa, S.C. (2021) Pancytopenia and thrombosis defects in zebrafish mutants of Fanconi anemia genes. Blood cells, molecules & diseases. 93:102640.
Progressive pancytopenia is a common feature observed in DNA crosslink repair deficiency disorder, Fanconi anemia (FA). However, this phenotype has not been recapitulated in single FA gene knockout animal models. In this study, we analyzed hematological characteristics in zebrafish null mutants for two FA genes, fanca and fanco. In adult mutants, we demonstrate age-associated reduction in blood cell counts for all lineages, resembling progressive pancytopenia in FA patients. In larval mutants, we demonstrate vascular injury-induced thrombosis defects, particularly upon treatment with crosslinking agent diepoxybutane (DEB), indicating DNA damage induced inefficiency of thrombocytes.
Genes / Markers
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Engineered Foreign Genes