Lanfranco Maia, Cacciottolo Rebecca, Borg Rebecca M, Vassallo Neville, Juge François, Bordonné Rémy, Cauchi Ruben J
Institut de Génétique Moléculaire de Montpellier, CNRS-UMR 5535, Université de Montpellier, France.
Department of Physiology and Biochemistry, Faculty of Medicine and Surgery, University of Malta, Msida, Malta.
FEBS Lett. 2017 Nov;591(21):3600-3614. doi: 10.1002/1873-3468.12853. Epub 2017 Oct 10.
The Spinal Muscular Atrophy disease protein Survival Motor Neuron (SMN) operates as part of a multiprotein complex whose components also include Gemins 2-8 and Unrip. The fruit fly Drosophila melanogaster is thought to have a slightly smaller SMN complex comprised of SMN, Gemin2/3/5 and, possibly, Unrip. Based upon in vivo interaction methods, we have identified novel interacting partners of the Drosophila SMN complex with homologies to Gemin4/6/7/8. The Gemin4 and Gemin8 orthologues are required for neuromuscular function and survival. The Gemin6/7/Unrip module can be recruited via the SMN-associated Gemin8, hence mirroring the human SMN complex architecture. Our findings lead us to propose that an elaborate SMN complex that is typical in metazoans is also present in Drosophila.
脊髓性肌萎缩症疾病蛋白存活运动神经元（SMN）作为一种多蛋白复合物的一部分发挥作用，该复合物的成分还包括Gemins 2 - 8和Unrip。果蝇被认为具有一个稍小的SMN复合物，由SMN、Gemin2/3/5以及可能的Unrip组成。基于体内相互作用方法，我们鉴定出了果蝇SMN复合物的新型相互作用伙伴，它们与Gemin4/6/7/8具有同源性。Gemin4和Gemin8的直系同源物是神经肌肉功能和存活所必需的。Gemin6/7/Unrip模块可以通过与SMN相关的Gemin8被招募，因此反映了人类SMN复合物的结构。我们通过研究提出，后生动物中典型的精细SMN复合物在果蝇中也存在。
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