PUBLICATION

Pancytopenia and thrombosis defects in zebrafish mutants of Fanconi anemia genes

Authors
Raman, R., Ramanagoudr-Bhojappa, R., Dhinoja, S., Ramaswami, M., Carrington, B., Jagadeeswaran, P., Chandrasekharappa, S.C.
ID
ZDB-PUB-220107-15
Date
2021
Source
Blood cells, molecules & diseases   93: 102640 (Journal)
Registered Authors
Chandrasekharappa, Settara, Jagadeeswaran, Pudur
Keywords
Fanconi anemia, Pancytopenia, Thrombocytopenia, Zebrafish knockouts
MeSH Terms
  • Animals
  • DNA Damage
  • Fanconi Anemia*/genetics
  • Humans
  • Pancytopenia*/genetics
  • Thrombosis*/genetics
  • Zebrafish
PubMed
34991062 Full text @ Blood Cells Mol. Dis.
Abstract
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
Figures
Show all Figures
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping