RRC ID 30820
著者 Faust JE, Manisundaram A, Ivanova PT, Milne SB, Summerville JB, Brown HA, Wangler M, Stern M, McNew JA.
タイトル Peroxisomes are required for lipid metabolism and muscle function in Drosophila melanogaster.
ジャーナル PLoS One
Abstract Peroxisomes are ubiquitous organelles that perform lipid and reactive oxygen species metabolism. Defects in peroxisome biogenesis cause peroxisome biogenesis disorders (PBDs). The most severe PBD, Zellweger syndrome, is characterized in part by neuronal dysfunction, craniofacial malformations, and low muscle tone (hypotonia). These devastating diseases lack effective therapies and the development of animal models may reveal new drug targets. We have generated Drosophila mutants with impaired peroxisome biogenesis by disrupting the early peroxin gene pex3, which participates in budding of pre-peroxisomes from the ER and peroxisomal membrane protein localization. pex3 deletion mutants lack detectible peroxisomes and die before or during pupariation. At earlier stages of development, larvae lacking Pex3 display reduced size and impaired lipid metabolism. Selective loss of peroxisomes in muscles impairs muscle function and results in flightless animals. Although, hypotonia in PBD patients is thought to be a secondary effect of neuronal dysfunction, our results suggest that peroxisome loss directly affects muscle physiology, possibly by disrupting energy metabolism. Understanding the role of peroxisomes in Drosophila physiology, specifically in muscle cells may reveal novel aspects of PBD etiology.
巻・号 9(6)
ページ e100213
公開日 2014-6-19
DOI 10.1371/journal.pone.0100213
PII PONE-D-14-09238
PMID 24945818
PMC PMC4063865
MeSH Animals Drosophila Proteins / genetics Drosophila Proteins / metabolism Drosophila melanogaster / metabolism* Gene Knockdown Techniques Lipid Metabolism* Muscles / physiology* Mutation / genetics Organ Specificity Peroxisomes / metabolism* Pupa / physiology RNA Interference
IF 2.74
引用数 16
WOS 分野 CELL BIOLOGY
リソース情報
ショウジョウバエ 6859R-4 DaG32 (DGRC#108252)