Faculty of Biology and Medicine at University of Lausanne and Lausanne University Hospital

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Published December 11, 2020 | Version v3
Dataset Restricted

Dataset of "ARP-T1 is a ciliogenesis protein associated with a novel ciliopathy in inherited basal cell cancer, Bazex-Dupré-Christol Syndrome"

Authors/Creators

  • 1. CHUV
  • 2. UNIL-CHUV
  • 3. University of Cologne
  • 4. CHU Bordeaux
  • 5. CHU Dijon
  • 6. INSERM

Description

This dataset is linked to the paper "ARP-T1 is a ciliogenesis protein associated with a novel ciliopathy in inherited basal cell cancer, Bazex-Dupré-Christol Syndrome". It contains all western-blot images and immunofluorescent confocal pictures related to this article. Actin-Related Protein-Testis1 (ARP-T1)/ ACTRT1 gene mutations cause the Bazex-Dupré-Christol Syndrome (BDCS) characterized by follicular atrophoderma, hypotrichosis and basal cell cancer. Here, we report an ARP-T1 interactome (deposit PXD016557) involved in ciliogenesis, endosomal recycling and septin ring formation. Consequently, ARP-T1 localizes to the midbody during cytokinesis and the basal body of primary cilia in G0. Tissue samples from BDCS patients show reduced ciliary length with significant correlations of ARP-T1 expression levels, confirmed by ACTRT1 knock down. We report that BDCS is a novel ciliopathy and the first case of a skin cancer ciliopathy, where ARP-T1 plays a critical role to prevent pathogenesis.

Notes

Modified deposit for a new submission

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