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ZMAT3 drives metabolic adaptation in Fanconi anemia, creating a preleukemic niche through sphingolipid reprogramming.

ABSTRACT: ZMAT3 alleviates cell death cells in Fanconi anemia via adaptation of sphingolipid metabolism.

ORGANISM(S): Mus musculus

PROVIDER: GSE309105 | GEO | 2026/06/24

REPOSITORIES: GEO

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ZMAT3 alleviates cell death in Fanconi anemia via adaptation of sphingolipid metabolism

Project description:Fanconi anemia (FA) is characterized by defective DNA repair and chronic p53 activation, predisposing to acute myeloid leukemia through persistent genomic instability. The molecular adaptations enabling cell survival under chronic stress remain poorly understood. This study investigates the role of ZMAT3, a p53 responsive RNA binding protein overexpressed in FA deficient cells, to elucidate its role in cellular adaptation. Using FA patient fibroblasts and Fancg KO mouse models, we launched transcriptomic profiling, lipidomic, and functional analyses. RNA-seq and lipidomic analyses revealed unexpected alterations in sphingolipid metabolism upon ZMAT3 depletion, linked to ceramide accumulation and downregulation of ASAH1 (acid ceramidase). Functionally, ZMAT3 depletion increased DNA damage and enhanced ferroptosis susceptibility, an iron dependent cell death mechanism. Mechanistically, our results showed that ZMAT3 promotes ASAH1 expression, limits ceramide accumulation and protects cells from ferroptosis induced death which could participate to preleukemic clonal evolution and pointing to areas for further exploration in FA-associated malignancies.

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ZMAT3 drives metabolic adaptation in Fanconi anemia, creating a preleukemic niche through sphingolipid reprogramming.

Project description:ZMAT3 drives metabolic adaptation in Fanconi anemia, creating a preleukemic niche through sphingolipid reprogramming.

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ZMAT3 alleviates cell death in Fanconi anemia via adaptation of sphingolipid metabolism

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Gene expression based identification of potential genes involved in Fanconi anemia

Project description:Fanconi anemia is a rare inherited hematological disorder which commonly presents with bone marrow failure, developmental abnormalities and susceptibility to cancer with high rates of prevalence in ethnic populations. The objective of this study was to identify potential genes that aid in the progression of the disease or produce its principal symptoms and to hypothesize enabling roles for certain genes that are not part of the central molecular machinery causing the disease. A total of 2 Fanconi anemia samples were collected from patients who displayed characteristic FA features. All of them gave positive results for the DNA breakage test after mitomycin C treatment. Samples were referred by Dr. Sheila Mohan of REFAIN (Registry for Fanconi anemia in India). Whole genome microarray analysis of peripheral blood from 2 patient samples and one normal individual. Sequential analysis of microarray data was carried out using gene ontology and pathway analysis to identify candidate genes.

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