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) |