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

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) that includes proteins involved in ciliogenesis, endosomal recycling and septin ring formation. In agreement, ARP-T1 localizes to the midbody during cytokinesis and the basal body of primary cilia in interphase. Tissue samples from ARP-T1-associated BDCS patients patients have reduced ciliary length. The severity of the shortened cilia significantly correlates with ARP-T1 levels, which was further validated by ACTRT1 knock down in culture cells. Thus, we propose that ARP-T1 participates in the regulation of cilia length and that ARP-T1-associated BDCS is a case of a skin cancer with ciliopathy characteristics.