Show simple item record

dc.contributor.advisor McNew, James A.
dc.contributor.advisor Bartel, Bonnie
dc.creatorFaust, Joseph
dc.date.accessioned 2014-08-26T21:19:55Z
dc.date.available 2014-08-26T21:19:55Z
dc.date.created 2013-12
dc.date.issued 2013-12-02
dc.date.submitted December 2013
dc.identifier.citation Faust, Joseph. "Peroxisome Biogenesis in Drosophila melanogaster: Protein Trafficking, Lipid Metabolism, and Muscle Function." (2013) Diss., Rice University. https://hdl.handle.net/1911/76723.
dc.identifier.urihttps://hdl.handle.net/1911/76723
dc.description.abstract Peroxisomes 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.mimetype application/pdf
dc.language.iso eng
dc.subjectPeroxisome biogenesis
Lipid Metabolism
dc.title Peroxisome Biogenesis in Drosophila melanogaster: Protein Trafficking, Lipid Metabolism, and Muscle Function
dc.contributor.committeeMember Diehl, Michael R.
dc.contributor.committeeMember Stern, Michael
dc.contributor.committeeMember Bennett, Matthew R.
dc.date.updated 2014-08-26T21:19:56Z
dc.type.genre Thesis
dc.type.material Text
thesis.degree.department Biochemistry and Cell Biology
thesis.degree.discipline Natural Sciences
thesis.degree.grantor Rice University
thesis.degree.level Doctoral
thesis.degree.name Doctor of Philosophy


Files in this item

Thumbnail

This item appears in the following Collection(s)

Show simple item record