PUBLICATION

Deficiency of Werner RecQ-type DNA helicase causes premature malnutrition in zebrafish

Authors
Ujibe, K., Kashima, M., Kataoka, M., Shimada, R., Okamoto, M., Kobayashi, I., Wada, S., Matsuda, H., Sakamoto, A., Hirata, H.
ID
ZDB-PUB-260224-7
Date
2026
Source
iScience   29: 114760114760 (Journal)
Registered Authors
Hirata, Hiromi, Kobayashi, Isao, Matsuda, Hiroki
Keywords
Developmental biology, Pathophysiology
Datasets
GEO:GSE276188
MeSH Terms
none
PubMed
41732282 Full text @ iScience
Abstract
Werner syndrome is a genetic progeria characterized by premature aging symptoms, but its early-onset pathology remains unclear. We generated wrn truncation mutant (wrn-/-) zebrafish using CRISPR/Cas9 and identified two premature mortality phases: 7-21 and 60-90 days post-fertilization (dpf). Time-course transcriptomics revealed two wrn-/- subgroups. One showed the reduced expression of intestinal and pancreatic exocrine genes at 7-9 dpf, while the other maintained normal expression initially but eventually showed reduced pancreatic exocrine genes by 21-35 dpf. The prematurely dying wrn-/- larvae exhibited intestinal villi and pancreatic defects, along with DNA damage, cell-cycle arrest, and apoptosis. They also had lower glycogen, glucose, and fat levels compared to wild-type and late-dying wrn-/- larvae, suggesting malnutrition. Notably, excess feeding partially improved their survival. These findings reveal early pathological features in the zebrafish model of Werner syndrome.
Genes / Markers
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Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping