INTRODUCTION: Congenital anomalies of the kidney and urinary tract (CAKUT) represent a group of structural and functional abnormalities that arise during the development of the kidneys and urinary tract in the embryonic period. With an incidence of one case per 500 newborns, these anomalies rank among the most common congenital disorders. CAKUT encompasses a broad spectrum of defects that can occur as isolated anomalies or as part of syndromic disorders, often leading to significant damage to the kidneys and urinary tract. Despite early diagnosis, the importance of CAKUT extends beyond the neonatal period, as it remains a leading cause of chronic kidney failure in children. Renal dysplasia and hypoplasia are responsible for approximately 20% of these cases, while hereditary diseases such as polycystic kidney disease account for 10% of chronic kidney failure in adults. AIM OF THE STUDY: This study uses immunofluorescence analysis to determine the spatial and temporal distribution of ITGA8 and VANGL2 proteins in the kidneys of healthy, normal, and CAKUT human fetal samples. Additionally, it aims to investigate the impact of Dab1 gene silencing on the expression of Itga8 and Vangl2 proteins in embryonic and postnatal mouse samples. MATERIALS AND METHODS: In this study, we analyzed the expression patterns of CAKUT candidate genes, integrin alpha-8 (ITGA8) and Van Gogh-like 2 (VANGL2), using immunohistochemistry and immunofluorescence in human fetal tissues of healthy and CAKUT-affected kidneys, and in the yotari (yot) mouse model carrying a Dab1 gene mutation. RESULTS: The results showed that the expression of Itga8 and Vangl2 varies under CAKUT conditions and is significantly elevated in embryonic kidneys of yotari (yot) mice compared to wild-type (wt) mice. At the same time, no significant differences were observed in postnatal kidneys. Spatially, Itga8 exhibited the strongest expression in the metanephric mesenchyme and renal vesicles/immature glomeruli, whereas Vangl2 demonstrated pronounced expression in the metanephric mesenchyme, vesicles, immature glomeruli, and collecting ducts. Additionally, VANGL2 maintained a constant expression in human samples during fetal maturation, while ITGA8 expression varied. Compared to healthy kidneys (CTRL), ITGA8 was poorly expressed in duplex kidneys (DK) and dysplastic kidneys (DYS). At the same time, VANGL2 showed significant expression in dysplastic kidneys (DYS) and lower expression in hypoplastic kidneys (HYP). CONCLUSION: These findings suggest that disruptions in ITGA8 and VANGL2 expression, caused by genetic factors such as Dab1 gene mutations, contribute to kidney developmental defects associated with CAKUT phenotypes. Identifying these proteins as potential prognostic markers and therapeutic targets for CAKUT opens opportunities for further research into the molecular mechanisms underlying the differential expression of ITGA8 and VANGL2.