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  1. KEEP
  2. Theses and Dissertations
  3. Barrett, The Honors College Thesis/Creative Project Collection
  4. Dysregulated ERK/MAPK Signaling in RASopathy Animal Model Systems Leads to a Decrease in mTOR Expression and Activation of Translational Machinery
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Dysregulated ERK/MAPK Signaling in RASopathy Animal Model Systems Leads to a Decrease in mTOR Expression and Activation of Translational Machinery

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Description

The RAS/MAPK (RAS/Mitogen Activated Protein Kinase) pathway is a highly conserved, canonical signaling cascade that is highly involved in cellular growth and proliferation as well as cell migration. As such, it plays an important role in development, specifically in development of the nervous system. Activation of ERK is indispensable for the differentiation of Embryonic Stem Cells (ESC) into neuronal precursors (Li z et al, 2006). ERK signaling has also shown to mediate Schwann cell myelination of the peripheral nervous system (PNS) as well as oligodendrocyte proliferation (Newbern et al, 2011). The class of developmental disorders that result in the dysregulation of RAS signaling are known as RASopathies. The molecular and cell-specific consequences of these various pathway mutations remain to be elucidated. While there is evidence for altered DNA transcription in RASopathies, there is little work examining the effects of the RASopathy-linked mutations on protein translation and post-translational modifications in vivo. RASopathies have phenotypic and molecular similarities to other disorders such as Fragile X Syndrome (FXS) and Tuberous Sclerosis (TSC) that show evidence of aberrant protein synthesis and affect related pathways. There are also well-defined downstream RAS pathway elements involved in translation. Additionally, aberrant corticospinal axon outgrowth has been observed in disease models of RASopathies (Xing et al, 2016). For these reasons, this present study examines a subset of proteins involved in translation and translational regulation in the context of RASopathy disease states. Results indicate that in both of the tested RASopathy model systems, there is altered mTOR expression. Additionally the loss of function model showed a decrease in rps6 activation. This data supports a role for the selective dysregulation of translational control elements in RASopathy models. This data also indicates that the primary candidate mechanism for control of altered translation in these modes is through the altered expression of mTOR.

Date Created
2017-05
Contributors
  • Hilbert, Alexander Robert (Author)
  • Newbern, Jason (Thesis director)
  • Olive, M. Foster (Committee member)
  • Bjorklund, Reed (Committee member)
  • School of Life Sciences (Contributor)
  • Barrett, The Honors College (Contributor)
Topical Subject
  • Cell Signaling
  • Neurobiology
  • RASopathies
Resource Type
Text
Extent
36 pages
Language
eng
Copyright Statement
In Copyright
Primary Member of
Barrett, The Honors College Thesis/Creative Project Collection
Series
Academic Year 2016-2017
Handle
https://hdl.handle.net/2286/R.I.43738
Level of coding
minimal
Cataloging Standards
asu1
System Created
  • 2017-10-30 02:50:58
System Modified
  • 2021-08-11 04:09:57
  •     
  • 1 year 9 months ago
Additional Formats
  • OAI Dublin Core
  • MODS XML

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