Characterization of connexin 43 protein concentration and localization in normal sheep and sheep affected with the CLN6 form of Batten disease: A dissertation submitted in partial fulfilment of the requirements for the Degree of Bachelor of Science with Honours

Abstract

The neuronal ceroid lipofuscinoses (NCLs, Batten disease) are fatal inherited neurodegenerative diseases characterized by gross brain atrophy, blindness, and intracellular accumulation oflysosome derived storage bodies. A CLN6 form in sheep is studied as a large animal model of the human diseases. This study describes connexin 43 protein concentration in general 6 month brain tissue as well as immunoreactive changes in the 6, 12 and 18 month parieto-occipital cortex from pre- and post-symptomatic affected sheep. Connexin 43 immunostaining seems to associate with glial activation found in affected sheep indicating connexin 43 immunoreactivity is likely contributed by gliosis and the potential upregulation of connexin 43 protein. Moreover, blood brain barrier connexin 43 could have accounted for connexin 43 immunoreactivity. Meanwhile, connexin 46 was found expressed in sheep brain may have a similar role to other connexins such as forming gap junctional communications.