Neuron-specific knockdown of the Drosophila fat induces reduction of life span, deficient locomotive ability, shortening of motoneuron terminal branches and defects in axonal targeting.
Identifieur interne : 000348 ( Main/Exploration ); précédent : 000347; suivant : 000349Neuron-specific knockdown of the Drosophila fat induces reduction of life span, deficient locomotive ability, shortening of motoneuron terminal branches and defects in axonal targeting.
Auteurs : Aya Nakamura [Japon] ; Ryo Tanaka [Japon] ; Kazushige Morishita [Japon] ; Hideki Yoshida [Japon] ; Yujiro Higuchi [Japon] ; Hiroshi Takashima [Japon] ; Masamitsu Yamaguchi [Japon]Source :
- Genes to cells : devoted to molecular & cellular mechanisms [ 1365-2443 ] ; 2017.
Abstract
Mutations in FAT4 gene, one of the human FAT family genes, have been identified in Van Maldergem syndrome (VMS) and Hennekam lymphangiectasia-lymphedema syndrome (HS). The FAT4 gene encodes a large protein with extracellular cadherin repeats, EGF-like domains and Laminin G-like domains. FAT4 plays a role in tumor suppression and planar cell polarity. Drosophila contains a human FAT4 homologue, fat. Drosophila fat has been mainly studied with Drosophila eye and wing systems. Here, we specially knocked down Drosophila fat in nerve system. Neuron-specific knockdown of fat shortened the life span and induced the defect in locomotive abilities of adult flies. In consistent with these phenotypes, defects in synapse structure at neuromuscular junction were observed in neuron-specific fat-knockdown flies. In addition, aberrations in axonal targeting of photoreceptor neuron in third-instar larvae were also observed, suggesting that fat involves in axonal targeting. Taken together, the results indicate that Drosophila fat plays an essential role in formation and/or maintenance of neuron. Both VMS and HS show mental retardation and neuronal defects. We therefore consider that these two rare human diseases could possibly be caused by the defect in FAT4 function in neuronal cells.
DOI: 10.1111/gtc.12500
PubMed: 28488382
Affiliations:
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xml:lang="en">Mutations in FAT4 gene, one of the human FAT family genes, have been identified in Van Maldergem syndrome (VMS) and Hennekam lymphangiectasia-lymphedema syndrome (HS). The FAT4 gene encodes a large protein with extracellular cadherin repeats, EGF-like domains and Laminin G-like domains. FAT4 plays a role in tumor suppression and planar cell polarity. Drosophila contains a human FAT4 homologue, fat. Drosophila fat has been mainly studied with Drosophila eye and wing systems. Here, we specially knocked down Drosophila fat in nerve system. Neuron-specific knockdown of fat shortened the life span and induced the defect in locomotive abilities of adult flies. In consistent with these phenotypes, defects in synapse structure at neuromuscular junction were observed in neuron-specific fat-knockdown flies. In addition, aberrations in axonal targeting of photoreceptor neuron in third-instar larvae were also observed, suggesting that fat involves in axonal targeting. Taken together, the results indicate that Drosophila fat plays an essential role in formation and/or maintenance of neuron. Both VMS and HS show mental retardation and neuronal defects. We therefore consider that these two rare human diseases could possibly be caused by the defect in FAT4 function in neuronal cells.</div></front></TEI><affiliations><list><country><li>Japon</li></country></list><tree><country name="Japon"><noRegion><name sortKey="Nakamura, Aya" sort="Nakamura, Aya" uniqKey="Nakamura A" first="Aya" last="Nakamura">Aya Nakamura</name></noRegion><name sortKey="Higuchi, Yujiro" sort="Higuchi, Yujiro" uniqKey="Higuchi Y" first="Yujiro" last="Higuchi">Yujiro Higuchi</name><name sortKey="Morishita, Kazushige" sort="Morishita, Kazushige" uniqKey="Morishita K" first="Kazushige" last="Morishita">Kazushige Morishita</name><name sortKey="Takashima, Hiroshi" sort="Takashima, Hiroshi" uniqKey="Takashima H" first="Hiroshi" last="Takashima">Hiroshi Takashima</name><name sortKey="Tanaka, Ryo" sort="Tanaka, Ryo" uniqKey="Tanaka R" first="Ryo" last="Tanaka">Ryo Tanaka</name><name sortKey="Yamaguchi, Masamitsu" sort="Yamaguchi, Masamitsu" uniqKey="Yamaguchi M" first="Masamitsu" last="Yamaguchi">Masamitsu Yamaguchi</name><name sortKey="Yoshida, Hideki" sort="Yoshida, Hideki" uniqKey="Yoshida H" first="Hideki" last="Yoshida">Hideki Yoshida</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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