Cargando…

A Motor Function for the DEAD-Box RNA Helicase, Gemin3, in Drosophila

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 functi...

Descripción completa

Detalles Bibliográficos
Autores principales: Cauchi, Ruben J., Davies, Kay E., Liu, Ji-Long
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2008
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2577925/
https://www.ncbi.nlm.nih.gov/pubmed/19023405
http://dx.doi.org/10.1371/journal.pgen.1000265
_version_ 1782160514794651648
author Cauchi, Ruben J.
Davies, Kay E.
Liu, Ji-Long
author_facet Cauchi, Ruben J.
Davies, Kay E.
Liu, Ji-Long
author_sort Cauchi, Ruben J.
collection PubMed
description 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.
format Text
id pubmed-2577925
institution National Center for Biotechnology Information
language English
publishDate 2008
publisher Public Library of Science
record_format MEDLINE/PubMed
spelling pubmed-25779252008-11-21 A Motor Function for the DEAD-Box RNA Helicase, Gemin3, in Drosophila Cauchi, Ruben J. Davies, Kay E. Liu, Ji-Long PLoS Genet Research Article 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. Public Library of Science 2008-11-21 /pmc/articles/PMC2577925/ /pubmed/19023405 http://dx.doi.org/10.1371/journal.pgen.1000265 Text en Cauchi et al. http://creativecommons.org/licenses/by/4.0/ 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 properly credited.
spellingShingle Research Article
Cauchi, Ruben J.
Davies, Kay E.
Liu, Ji-Long
A Motor Function for the DEAD-Box RNA Helicase, Gemin3, in Drosophila
title A Motor Function for the DEAD-Box RNA Helicase, Gemin3, in Drosophila
title_full A Motor Function for the DEAD-Box RNA Helicase, Gemin3, in Drosophila
title_fullStr A Motor Function for the DEAD-Box RNA Helicase, Gemin3, in Drosophila
title_full_unstemmed A Motor Function for the DEAD-Box RNA Helicase, Gemin3, in Drosophila
title_short A Motor Function for the DEAD-Box RNA Helicase, Gemin3, in Drosophila
title_sort motor function for the dead-box rna helicase, gemin3, in drosophila
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2577925/
https://www.ncbi.nlm.nih.gov/pubmed/19023405
http://dx.doi.org/10.1371/journal.pgen.1000265
work_keys_str_mv AT cauchirubenj amotorfunctionforthedeadboxrnahelicasegemin3indrosophila
AT davieskaye amotorfunctionforthedeadboxrnahelicasegemin3indrosophila
AT liujilong amotorfunctionforthedeadboxrnahelicasegemin3indrosophila
AT cauchirubenj motorfunctionforthedeadboxrnahelicasegemin3indrosophila
AT davieskaye motorfunctionforthedeadboxrnahelicasegemin3indrosophila
AT liujilong motorfunctionforthedeadboxrnahelicasegemin3indrosophila