a motor function for the dead-box rna helicase, gemin3, in drosophila出局区rna解旋酶的运动机能,gemin3果蝇.pdfVIP

A Motor Function for the DEAD-Box RNA Helicase, Gemin3, in Drosophila Ruben J. Cauchi, Kay E. Davies, Ji-Long Liu* Medical Research Council Functional Genomics Unit, Department of Physiology, Anatomy, and Genetics, University of Oxford, Oxford, United Kingdom Abstract The survival motor neuron (SMN) protein, the determining factor for spinal muscular atrophy (SMA), is complexed with a group of proteins in human cells. Gemin3 is the only RNA helicase in the SMN complex. Here, we report the identification of Drosophila melanogaster Gemin3 and investigate its function in vivo. Like in vertebrates, Gemin3 physically interacts with SMN in Drosophila. Loss of function of gemin3 results in lethality at larval and/or prepupal stages. Before they die, gemin3 mutant larvae exhibit declined mobility and expanded neuromuscular junctions. Expression of a dominant-negative transgene and knockdown of Gemin3 in mesoderm cause lethality. A less severe Gemin3 disruption in developing muscles leads to flightless adults and flight muscle degeneration. Our findings suggest that Drosophila Gemin3 is required for larval development and motor function. Citation: Cauchi RJ, Davies KE, Liu J-L (2008) A Motor Function for the DEAD-Box RNA Helicase, Gemin3, in Drosophila. PLoS Genet 4(11): e1000265. doi:10.1371/ journal.pgen.1000265 Editor: Gregory A. Cox, The Jackson Laboratory, United States of America Received July 14, 2008; Accepted October 16, 2008; Published November 21, 2008 Copyright: 2008 Cauchi et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Funding: This work was supported by the Medical Research Council. Competing Interests: The authors have decl