Peroxisome Biogenesis in Drosophila melanogaster: Protein Trafficking, Lipid Metabolism, and Muscle Function

Simple item page
dc.contributor.advisorMcNew, James A.
dc.contributor.advisorBartel, Bonnie
dc.contributor.committeeMemberDiehl, Michael R.
dc.contributor.committeeMemberStern, Michael
dc.contributor.committeeMemberBennett, Matthew R.
dc.creatorFaust, Joseph
dc.date.accessioned2014-08-26T21:19:55Z
dc.date.available2014-08-26T21:19:55Z
dc.date.created2013-12
dc.date.issued2013-12-02
dc.date.submittedDecember 2013
dc.date.updated2014-08-26T21:19:56Z
dc.description.abstractPeroxisomes are ubiquitous organelles required for many essential functions, such as fatty acid metabolism. Defects in peroxisome biogenesis cause a spectrum of human diseases known as peroxisome biogenesis disorders (PBDs). These devastating diseases lack effective therapies and it is unclear how peroxisome dysfunction causes the disease state. Animal models are needed to understand the connection between peroxisome biology and animal physiology. The fruit fly, Drosophila melanogaster, has recently become an important animal model in the study of peroxisomes. We have identified the major peroxisomal proteins and pathways in flies and examined peroxisomal protein trafficking. We have found that fruit fly peroxisomes share many features in common with higher animals, but display some important differences. Flies appear to have lost one of the pathways used in other organisms to target proteins to the peroxisomal matrix. Also some proteins are dually localized to peroxisomes and the cytoplasm likely through a weak interaction with the protein machinery that brings peroxisomal proteins into the organelle. We have also generated fly mutants with impaired peroxisome biogenesis and shown that peroxisomes are required for normal development and lipid metabolism. Flies with impaired peroxisome biogenesis also show defects in multiple processes that depend on muscle function, such as locomotion. PBD patients also display muscle defects, but it is thought to be a secondary effect of neuronal dysfunction. We propose that peroxisome loss in humans, like in flies, may directly affect muscle physiology, possibly by disrupting energy metabolism. Understanding the role of peroxisomes in fly physiology and specifically in muscle cells may reveal novel aspects of PBD etiology.
dc.format.mimetypeapplication/pdf
dc.identifier.citationFaust, Joseph. "Peroxisome Biogenesis in Drosophila melanogaster: Protein Trafficking, Lipid Metabolism, and Muscle Function." (2013) Diss., Rice University. <a href="https://hdl.handle.net/1911/76723">https://hdl.handle.net/1911/76723</a>.
dc.identifier.urihttps://hdl.handle.net/1911/76723
dc.language.isoeng
dc.rightsCopyright is held by the author, unless otherwise indicated. Permission to reuse, publish, or reproduce the work beyond the bounds of fair use or other exemptions to copyright law must be obtained from the copyright holder.
dc.subjectPeroxisome biogenesis
dc.subjectLipid Metabolism
dc.titlePeroxisome Biogenesis in Drosophila melanogaster: Protein Trafficking, Lipid Metabolism, and Muscle Function
dc.typeThesis
dc.type.materialText
thesis.degree.departmentBiochemistry and Cell Biology
thesis.degree.disciplineNatural Sciences
thesis.degree.grantorRice University
thesis.degree.levelDoctoral
thesis.degree.nameDoctor of Philosophy
Files
Original bundle
Now showing 1 - 1 of 1
Thumbnail Image
Name:
Joseph Faust - Thesis with Signatures.pdf
Size:
6.18 MB
Format:
Adobe Portable Document Format
Download
License bundle
Now showing 1 - 1 of 1
No Thumbnail Available
Name:
license.txt
Size:
941 B
Format:
Plain Text
Description:
Download
Collections
Rice University Electronic Theses and Dissertations