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Structure-function Studies in Mouse Embryonic Stem Cells Using Recombinase-mediated Cassette Exchange

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ERCC1 is required for FANCD2 focus formation.

Kevin M McCabe¹, Aaron Hemphill, Yassmine Akkari

¹Department of Molecular and Medical Genetics, Oregon Health and Science University, Portland, OR 97239, USA.

Molecular Genetics and Metabolism

|August 2, 2008

Summary

Excision repair cross complementing protein 1 (ERCC1) is crucial for Fanconi anemia pathway activation. ERCC1 facilitates FANCD2 monoubiquitination and nuclear foci formation after DNA double-strand breaks occur.

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Area of Science:

Molecular Biology
Genetics
DNA Repair Mechanisms

Background:

Fanconi anemia (FA) is a rare genetic disorder characterized by DNA interstrand crosslink sensitivity and genomic instability.
Excision repair cross complementing protein 1 (ERCC1) is known for its role in nucleotide excision repair and potentially in crosslink repair.

Purpose of the Study:

To investigate the relationship between ERCC1 and the Fanconi anemia DNA repair pathway.
To determine ERCC1's specific role in FA pathway activation following DNA damage.

Main Methods:

Utilized siRNA to deplete ERCC1 levels in normal human fibroblasts and Fanconi anemia patient-derived fibroblasts.
Assessed DNA double-strand break formation via gammaH2AX.
Monitored FANCD2 monoubiquitination and nuclear foci formation in response to mitomycin C (MMC) and hydroxyurea (HU) treatments.

Main Results:

ERCC1 depletion did not impede double-strand break formation after crosslink damage.
However, ERCC1 deficiency significantly reduced FANCD2 monoubiquitination and abolished FANCD2 nuclear foci formation upon MMC treatment.
ERCC1 was also essential for FANCD2 nuclear foci localization when DNA replication was arrested by hydroxyurea.

Conclusions:

ERCC1 plays a critical role downstream of double-strand break formation in the Fanconi anemia pathway.
ERCC1 is required for the complete activation of the FA pathway, specifically for proper FANCD2 processing and localization.