Singh et al, 2006 used RNA interference to decrease the expression of Drosophila BHD homologue (DBHDH) and showed that DBHDH was required for male germline stem cells (GSC) maintenance in the fly testis. Subsequent investigation suggested that DBHDH regulates GSC maintenance downstream of, or in parallel to the Janus kinase-signal transducers and activators of transcription (JAK/STAT) and Decapentaplegic (Dpp) signal-transduction pathways. JAK-STAT signalling cascade regulates stem cell renewal or differentiation of renal stem cells whilst the Dpp signal-transduction pathway is crucial for polarisation of cell differentiation in wing development.

Further studies into the Drosophila model by Singh and Hou (2009) have shown that over-expression of JAK-STAT signalling results in enlargement of Malphigian Tubules i.e. the Drosphila equivalent of kidneys, coupled with an increase in proliferating cells, mitotically active cells and renal stem cells. Aberrant signalling of JAK-STAT signalling could contribute to renal cystic or carcinoma phenotype.

Further details: Lab essentials – BHD Animal Models

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