Relationship Between Bicaudal-C And Pkd

Research Article
Ao Li
Bicaudal-C; Bicc1; jcpk; bpk; 67Gso; polycystic kidney disease; mouse models

Translational activation and repression play an important role in the spatial-temporal regulation of gene expression in embryonic development. Bicaudal-C of Drosophila is an RNA-binding molecule believed to function at this post-transcriptional level. Loss-of-function mutants in Drosophila affects anterior-posterior patterning, producing abnormal embryos lacking head formation and with duplicate posterior segments. Mouse Bic-C gene(Bicc1) is localized to a C1 region located on Chromosome 10. Bicaudal-C contains several conserved N-terminal KH domains and a conserved C-terminal SAM domain. The KH domains have been shown to bind RNA, and the SAM domain is thought to play a role in protein-protein interactions. There are three mouse mutant models for Bicc1,jcpk,bpkand 67Gso. Mutations in different sites of Bicc1 in these models produce the cystic phenotypes in the kidney and affect the normal formation/functions of other organs(liver, pancreas etc.), which are very similar to human polycystic kidney disease(PKD). Moreover, mutation of Bicaudal-C in other species also causes polycystic kidney disease-like phenotypes.Furthermore, emerging evidence also indicates thatnormal Bicc1 expression requires ADPKD causal gene expression. In this review, we outline the recent progress in Bicaudal-C research and its relationship with polycystic kidney disease.