Transcriptome analysis of nphp1(ex15-del4); nphp4(sa38686) double mutant zebrafish

Nephronophthisis (NPH) is the most common genetic cause of end-stage renal disease in children and young adults. Mutations in more than 25 genes, called NPHPs, were found to be causative. We performed RNA sequencing analysis to investigate the transcriptional changes in nphp1(ex15-del4); nphp4(sa38686) homozygous mutant zebrafish compared to wild-type siblings. Our analysis revealed significant alterations in gene expression profiles associated with cilia function, cell signaling pathways, and kidney development. This dataset provides insights into the molecular mechanisms underlying NPH pathogenesis and genetic compensation in a zebrafish model of the disease. Overall design: RNA-seq profiling of nphp1(ex15-del4); nphp4(sa38686) double mutant zebrafish and their wild-type siblings at 48 hpf. Four biological replicates were sequenced for each genotype.