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We previously showed that expression of patient variants in Dishevelled 1 (DVL1) in\n                      <jats:italic>Drosophila<\/jats:italic>\n                      and chicken models disrupts the balance of canonical and non\u2010canonical Wnt signaling.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      In this study, we further examine morphological changes that occur due to expression of\n                      <jats:italic>DVL1<\/jats:italic>\n                      <jats:sup>\n                        <jats:italic>1519\u0394T<\/jats:italic>\n                      <\/jats:sup>\n                      , which serves as a prototype for other pathogenic variants. We show that epithelial imaginal disc development is disrupted in legs and wings and accompanied by increased cell death, without changes in cell proliferation. By inhibiting caspase\u2010dependent cell death, we show that the altered epithelial morphology is not solely due to variant\u2010induced cell death. Furthermore, we find alterations of basement membrane components and modulators. Notably we find ectopic Mmp1 expression and tissue distortion, which is dependent on JNK signaling. We also find an abnormal abundance of\n                      <jats:italic>Drosophila<\/jats:italic>\n                      collagen IV (Viking) in pupal wing development. Due to the complex nature of appendage development, we also examined the Bone Morphogenetic Protein pathway and found elevated signaling activity via the transcriptional readou\n                      <jats:italic>t dad\u2010lacZ<\/jats:italic>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      Through these studies, we have gained more insight into the developmental consequences of\n                      <jats:italic>DVL1<\/jats:italic>\n                      variants implicated in autosomal dominant Robinow syndrome.\n                    <\/jats:p>\n                  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