The Role of Cux1 on Ift122 in Autosomal Dominant Polycystic Kidney Disease

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Authors
Thaj, Omar
Issue Date
2022-01-01
Type
Thesis
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en_US
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Abstract
Polycystic kidney disease (PKD) is a common genetic disorder that causes the formation of fluid filled cysts in the kidney which eventually leads to renal failure. Autosomal dominant PKD (ADPKD) is the more common form of the fatal disease, and results from a mutation in either the PKD1 or PKD2 gene which respectfully encode for polycystin-1 and polycystin-2. These proteins are located in the membrane of renal nephron primary cilium, an important structure that plays a crucial role in regulating cell proliferation and differentiation. Located in the lumen of epithelial cells, they are thought to play a role in PKD cystogenesis. Cux1 is a transcription protein that regulates cell growth and cystic progression in PKD. New research has shown that Cux1 has a role in regulating ciliary genes such as Ift122 in the Galapagos Cormorant bird. The Ift122 protein is used in ciliary formation and maintenance. This study aimed to observe whether Cux1 has a role in regulating the Ift122 gene in mammals as was seen in other species. Ift122 protein expression was measured in this experiment by double labeling kidney tissue sections from various genotype RBP-J knockout mouse models. RBP-J is part of the notch signaling pathway, and when knocked out causes an increase in Cux1. The results found that in upregulated Cux1 environments, Ift122 protein expression was absent. This suggests that Cux1 plays a role in the downregulation of the ciliary protein Ift122 in mammals. As a change in Ift122 protein expression can cause ciliary disfunction, this study suggests another way that Cux1 is involved in the PKD ciliopathy. The data can be used with future experiments to identify therapeutic targets for disease treatment.
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iv, 21 p.
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Kalamazoo College
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U.S. copyright laws protect this material. Commercial use or distribution of this material is not permitted without prior written permission of the copyright holder.
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