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	Drosophila models of peroxisomal biogenesis disorder: peroxins are required for spermatogenesis and very-long-chain fatty acid metabolism
	Chen, Haiyang1,2; Liu, Zhonghua1; Huang, Xun1
刊名	Human molecular genetics
	2010-02-01
卷号	19期号:3页码:494-505
ISSN号	0964-6906
DOI	10.1093/hmg/ddp518
通讯作者	Huang, xun(xhuang@genetics.ac.cn)
英文摘要	Peroxisomes are vital eukaryotic organelles that participate in lipid metabolism, in particular the metabolism of very-long-chain fatty acids (vlcfa). the biogenesis of peroxisomes is regulated by a set of peroxin proteins (pex). in humans, mutations affecting peroxin protein production or function result in devastating diseases classified as peroxisome biogenesis disorders (pbd). the way in which peroxisomal dysfunction leads to the pathophysiological consequences of pbd is not well understood. here we report that drosophila pex mutants faithfully recapitulate several key features of human pbd, including impaired peroxisomal protein import, elevated vlcfa levels and growth retardation. moreover, disruption of pex function results in spermatogenesis defects, including spermatocyte cytokinesis failure in drosophila. importantly, increased vlcfa levels enhance these spermatogenesis defects whereas reduced vlcfa levels alleviate them. thus, regulation of proper vlcfa levels by pex genes is crucial for spermatogenesis. together our study reveals an indispensable function of pex genes during spermatogenesis and provides a causative link between the phenotypic severity of pex mutants and vlcfa levels.
WOS关键词	ZELLWEGER-SYNDROME ; SPERMATID DIFFERENTIATION ; PROTEIN IMPORT ; CYTOKINESIS ; MEMBRANE ; HOMOLOG ; GENE ; FERTILITY ; MEIOSIS ; TWINE
WOS研究方向	Biochemistry & Molecular Biology ; Genetics & Heredity
WOS类目	Biochemistry & Molecular Biology ; Genetics & Heredity
语种	英语
出版者	OXFORD UNIV PRESS
WOS记录号	WOS:000273227200009
内容类型	期刊论文
URI标识	http://www.corc.org.cn/handle/1471x/2409301
专题	中国科学院大学
通讯作者	Huang, Xun
作者单位	1.Chinese Acad Sci, Inst Genet & Dev Biol, Mol & Dev Biol Lab, Beijing 100101, Peoples R China 2.Chinese Acad Sci, Grad Univ, Beijing 100049, Peoples R China
推荐引用方式 GB/T 7714	Chen, Haiyang,Liu, Zhonghua,Huang, Xun. Drosophila models of peroxisomal biogenesis disorder: peroxins are required for spermatogenesis and very-long-chain fatty acid metabolism[J]. Human molecular genetics,2010,19(3):494-505.
APA	Chen, Haiyang,Liu, Zhonghua,&Huang, Xun.(2010).Drosophila models of peroxisomal biogenesis disorder: peroxins are required for spermatogenesis and very-long-chain fatty acid metabolism.Human molecular genetics,19(3),494-505.
MLA	Chen, Haiyang,et al."Drosophila models of peroxisomal biogenesis disorder: peroxins are required for spermatogenesis and very-long-chain fatty acid metabolism".Human molecular genetics 19.3(2010):494-505.

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