Congenital anomalies of the kidney and urinary tract (CAKUT) account for 23% of congenital anomalies at birth and are implicated in roughly 50% of pediatric cases and 7% of cases of end-stage kidney disease in adults globally. The development, maturation, and morphogenesis of kidneys are intricate processes influenced by numerous genes. So far, monogenic abnormalities linked with CAKUT involve mutations in over 30 genes, identified in a limited proportion (12%) of examined cases, with insufficient evidence to establish their direct causal relationship. Previously conducted research has shown that Fibroblast Growth Factor Receptors 1 and 2 (FGFR1 and FGFR2) and Receptor-interacting protein kinase 5 (RIP5) are potential candidate genes that may lead to the development of congenital anomalies of the kidneys and urinary system, either in a syndromic or isolated form. Hence, the objective of this study was to examine the spatial and temporal distribution of the specified proteins in the yotari (Dab1-/-) mouse model, as well as in normally developing human kidneys and those affected by CAKUT (including duplex (DU) kidneys, hypoplastic kidneys (HYP), and dysplastic kidneys (DYS)), spanning from the 22nd to the 41st week of fetal development. This investigation aimed to discern the potential correlation between gene and protein expression alterations and the onset of CAKUT. Utilizing the Dab1-/- mouse model, we identified notable discrepancies in the spatio- temporal expression profiles of FGFR1, FGFR2, and RIP5 during embryonic and postnatal developmental phases. The consistent expression of these proteins across all examined stages of kidney development suggests their critical involvement in the early differentiation of the metanephrogenic mesoderm, ureteric bud branching, nephrogenesis, and the viability of nephron progenitors. Given their expression in various segments of the nephron and some within the renal vasculature, it is reasonable to speculate about their significance in maintaining overall kidney homeostasis and maturation, as well as their potential role in the genesis of diverse renal pathologies. The precise mechanism through which the Reelin/Dab1 signalling pathway influences the expression of the examined markers remains unclear. The reduction in Erk1/2 and mTOR expression in the kidneys of yotari mice may be attributed to a proposed signaling pathway where Dab1 is only partially involved in MEK/Erk1/2 activation mediated by Reelin. The impact of CAKUT candidate genes on nephrogenesis and renal function in normally developing human kidneys and those affected by CAKUT is supported by significant variations in the spatio-temporal expression patterns of the investigated markers. The elevated expression of FGFR1 and FGFR2 mRNA and proteins, particularly in the cytoplasmic and nuclear regions of CAKUT-affected kidney cells compared to normal tissue, along with altered dynamics in the expression of the examined proteins, suggests that activation of the FGF signaling pathway plays a crucial role in the pathogenesis of congenital kidney anomalies and urinary tract issues. The decreased expression of RIP5 during the developmental periods under investigation cannot solely be attributed to the expected levels of apoptosis in hypoplastic and dysplastic kidneys, indicating that other signaling pathways regulate the apoptosis process. Our findings underscore the pivotal role of the investigated markers in normal kidney development and their potential significance in renal pathology and diagnostics, where they could serve as biomarkers and therapeutic targets. Further research is warranted to delineate their roles in regulating renal differentiation, thereby enhancing personalized medicine approaches for congenital kidney and urinary tract anomalies.