Degree Type
Honors Capstone Project
Date of Submission
Spring 5-1-2019
Capstone Advisor
Jessica MacDonald
Honors Reader
James Hewett
Capstone Major
Biology
Capstone College
Arts and Science
Audio/Visual Component
no
Capstone Prize Winner
no
Won Capstone Funding
yes
Honors Categories
Sciences and Engineering
Subject Categories
Biology | Life Sciences | Neuroscience and Neurobiology
Abstract
The severe neurodevelopmental disorder Rett syndrome (RTT) primarily affects girls. Girls with RTT seem to develop normally until 6-18 months when they undergo a period of a regression where they lose previously acquired functions. RTT is the second most leading cause of intellectual disability in girls. Symptoms commonly associated with RTT, include loss of speech and motor function as well as respiratory problems, distinct hand wringing, and autistic characteristics. RTT is caused by mutations in the X-linked gene methyl-CpG-binding protein 2 (Mecp2). Both RTT patients and Mecp2 deficient mouse models have decreased dendritic complexity and reduced soma size of neurons within the cerebral cortex. Mecp2 was thought to primarily function in mature neurons and not progenitors and early developing neurons, which has led to an overwhelming focus of RTT research on post-symptomatic mice. However, there is expanding evidence that Mecp2 is important for early brain development, in addition to mature neuronal function. Therefore, we investigated the role Mecp2 plays in neuronal development by assessing neocortical development in pre-symptomatic postnatal day 6 (P6) Mecp2 null male (-/y) mice. We used immunohistochemistry with known neocortical layer-specific and areal specific molecular markers to determine if the laminar formation of the neocortex and precise formation of functional areas are disrupted. We found that the somatosensory area of the neocortex is significantly increased in the Mecp2 null mice compared with wildtype littermates, but the overall formation of the neocortical layers is not altered. Additional experiments are currently underway to establish whether Mecp2 function is necessary for the maturation of neocortical projection neurons and their subtype identity.
Recommended Citation
Ortiz, Camerin, "Investigation of the role of MeCP2 in the precise laminar and areal development of neocortical neurons in a Rett Syndrome mouse model" (2019). Renée Crown University Honors Thesis Projects - All. 1338.
https://surface.syr.edu/honors_capstone/1338
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This work is licensed under a Creative Commons Attribution 4.0 International License.
