A Shortdrome : An Analysis Of Alagille Syndrome

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Mutations in genes coding for component of the Notch signaling pathway cause a disease called Alagille syndrome. Alagille syndrome (ALGS) is a multi-organ pediatric disorder. Some ALGS patients develop small multi-cystic kidney disease. The Surendran lab has developed mouse models of Notch-signaling-deficient multicystic kidney disease. Studies in the mouse models have revealed that Notch signaling “maintains the alignment of cell division in the proximal tubules during nephrogenesis and loss of Notch signaling may lead to cystic renal disease and tumorigenesis due to misoriented cell division” (Surendran, Selassie, Liapis, Krigman, Kopan, 2010). Notch Signaling occurs between neighboring cells, in which ligand expressing cell activate the …show more content…

To be able to tell whether there are cysts forming, comparing the wild type and mutants is important to do. Genotyping is the first step to tell which mice are wild types and which are mutants, which is something I personally have done. The members of my lab are the ones that then harvest the kidneys from the mice. Next, tissue blocks are made with paraffin wax, which is used to embed the kidneys and then are mounted onto glass slides. Thin sections of tissues are attached to individual glass slides, where multiple sections can be arranged on one glass slide. Once the mounting is complete, sections are dried in preparation for paraffinization. To start staining, the paraffin must be completely removed, which we use xylene. We have to leave the slides in xylene overnight to de-paraffinize the slides and then we can start the washing cycle the next day. I have to use appropriately-labeled antibodies so they can specifically bind to their target antigens, so that I can localize specific cellular components within cells and within their histological context. Both primary and secondary antibodies are diluted into buffer that helps to stabilize the antibody and to discourage nonspecific binding. It is important to rinse the slides in-between antibody applications to remove

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