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1
α-COP binding to the survival motor neuron protein SMN is required for neuronal process outgrowth.α-COP与生存运动神经元蛋白SMN的结合是神经元突起生长所必需的。
Hum Mol Genet. 2015 Dec 20;24(25):7295-307. doi: 10.1093/hmg/ddv428. Epub 2015 Oct 13.
2
Dilysine motifs in exon 2b of SMN protein mediate binding to the COPI vesicle protein α-COP and neurite outgrowth in a cell culture model of spinal muscular atrophy.SMN 蛋白外显子 2b 中的二赖氨酸基序介导与 COPI 囊泡蛋白 α-COP 的结合，并在脊髓性肌萎缩症的细胞培养模型中促进神经突生长。
Hum Mol Genet. 2013 Oct 15;22(20):4043-52. doi: 10.1093/hmg/ddt254. Epub 2013 May 31.
3
Interaction between alpha-COP and SMN ameliorates disease phenotype in a mouse model of spinal muscular atrophy.α-COP 与 SMN 相互作用可改善脊髓性肌萎缩症小鼠模型的疾病表型。
Biochem Biophys Res Commun. 2019 Jun 25;514(2):530-537. doi: 10.1016/j.bbrc.2019.04.176. Epub 2019 May 3.
4
Abnormal Golgi morphology and decreased COPI function in cells with low levels of SMN.在 SMN 水平较低的细胞中，高尔基形态异常，COPI 功能下降。
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5
The COPI vesicle complex binds and moves with survival motor neuron within axons.COPI 囊泡复合物与生存运动神经元在轴突内结合并移动。
Hum Mol Genet. 2011 May 1;20(9):1701-11. doi: 10.1093/hmg/ddr046. Epub 2011 Feb 7.
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Chondrolectin affects cell survival and neuronal outgrowth in in vitro and in vivo models of spinal muscular atrophy.软骨凝集素影响脊髓性肌萎缩症的体外和体内模型中的细胞存活和神经元生长。
Hum Mol Genet. 2014 Feb 15;23(4):855-69. doi: 10.1093/hmg/ddt477. Epub 2013 Sep 25.
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Decreased Motor Neuron Support by SMA Astrocytes due to Diminished MCP1 Secretion.由于MCP1分泌减少，脊髓性肌萎缩症星形胶质细胞对运动神经元的支持作用降低。
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HuD and the Survival Motor Neuron Protein Interact in Motoneurons and Are Essential for Motoneuron Development, Function, and mRNA Regulation.HuD与生存运动神经元蛋白在运动神经元中相互作用，对运动神经元的发育、功能及mRNA调节至关重要。
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Modeling the differential phenotypes of spinal muscular atrophy with high-yield generation of motor neurons from human induced pluripotent stem cells.通过从人类诱导多能干细胞高效生成运动神经元来模拟脊髓性肌萎缩症的不同表型。
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The spinal muscular atrophy disease protein SMN is linked to the Rho-kinase pathway via profilin.脊髓性肌萎缩症相关蛋白 SMN 通过原肌球蛋白与 Rho-激酶通路相连接。
Hum Mol Genet. 2011 Dec 15;20(24):4865-78. doi: 10.1093/hmg/ddr425. Epub 2011 Sep 14.

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1
Sm-site containing mRNAs can accept Sm-rings and are downregulated in Spinal Muscular Atrophy.含有Sm位点的mRNA可接受Sm环，且在脊髓性肌萎缩症中表达下调。
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Conditional knockout mouse model demonstrates that Copa expression is required for viability in development and adulthood.条件性基因敲除小鼠模型表明，Copa表达对于发育和成年期的生存能力是必需的。
Biochem Biophys Res Commun. 2025 Aug 30;776:152201. doi: 10.1016/j.bbrc.2025.152201. Epub 2025 Jun 14.
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Hepatocyte-intrinsic SMN deficiency drives metabolic dysfunction and liver steatosis in spinal muscular atrophy.肝细胞内生存运动神经元（SMN）缺乏导致脊髓性肌萎缩症中的代谢功能障碍和肝脂肪变性。
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COPI coatomer subunit α-COP interacts with the RNA binding protein Nucleolin via a C-terminal dilysine motif.COP 衣被复合体亚基 α-COP 通过一个 C 末端双赖氨酸基序与 RNA 结合蛋白核仁蛋白相互作用。
Hum Mol Genet. 2023 Nov 17;32(23):3263-3275. doi: 10.1093/hmg/ddad140.
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Spinal Muscular Atrophy Patient iPSC-Derived Motor Neurons Display Altered Proteomes at Early Stages of Differentiation.脊髓性肌萎缩症患者诱导多能干细胞衍生的运动神经元在分化早期显示蛋白质组改变。
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What Genetics Has Told Us and How It Can Inform Future Experiments for Spinal Muscular Atrophy, a Perspective.遗传学告诉了我们什么，以及它如何为未来的脊髓性肌萎缩症实验提供信息：一个视角。
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Sumoylation regulates the assembly and activity of the SMN complex.SUMOylation 调节 SMN 复合物的组装和活性。
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8
Mutations in the COPI coatomer subunit α-COP induce release of Aβ-42 and amyloid precursor protein intracellular domain and increase tau oligomerization and release.α-COP 衣被复合体亚基突变可诱导 Aβ-42 和淀粉样前体蛋白细胞内域的释放，并增加 tau 寡聚化和释放。
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Local Secretory Trafficking Pathways in Neurons and the Role of Dendritic Golgi Outposts in Different Cell Models.神经元中的局部分泌运输途径以及树突状高尔基体驻所在不同细胞模型中的作用
Front Mol Neurosci. 2020 Nov 26;13:597391. doi: 10.3389/fnmol.2020.597391. eCollection 2020.
10
Conditional deletion of SMN in cell culture identifies functional SMN alleles.在细胞培养中条件性缺失 SMN 可鉴定功能性 SMN 等位基因。
Hum Mol Genet. 2020 Nov 1;29(21):3477-3492. doi: 10.1093/hmg/ddaa229. Epub 2020 Oct 19.

本文引用的文献

1
Astrocytes influence the severity of spinal muscular atrophy.星形胶质细胞影响脊髓性肌萎缩症的严重程度。
Hum Mol Genet. 2015 Jul 15;24(14):4094-102. doi: 10.1093/hmg/ddv148. Epub 2015 Apr 24.
2
Splicing changes in SMA mouse motoneurons and SMN-depleted neuroblastoma cells: evidence for involvement of splicing regulatory proteins.脊髓性肌萎缩症小鼠运动神经元和SMN缺失的神经母细胞瘤细胞中的剪接变化：剪接调节蛋白参与的证据
RNA Biol. 2014;11(11):1430-46. doi: 10.1080/15476286.2014.996494.
3
Presynaptic localization of Smn and hnRNP R in axon terminals of embryonic and postnatal mouse motoneurons.胚胎期和出生后小鼠运动神经元轴突终末中运动神经元存活蛋白（Smn）和不均一核糖核蛋白R（hnRNP R）的突触前定位。
PLoS One. 2014 Oct 22;9(10):e110846. doi: 10.1371/journal.pone.0110846. eCollection 2014.
4
Motoneuron development influences dorsal root ganglia survival and Schwann cell development in a vertebrate model of spinal muscular atrophy.在脊髓性肌萎缩症的脊椎动物模型中，运动神经元发育影响背根神经节存活和雪旺细胞发育。
Hum Mol Genet. 2015 Jan 15;24(2):346-60. doi: 10.1093/hmg/ddu447. Epub 2014 Sep 1.
5
Molecular mechanisms and animal models of spinal muscular atrophy.脊髓性肌萎缩症的分子机制与动物模型
Biochim Biophys Acta. 2015 Apr;1852(4):685-92. doi: 10.1016/j.bbadis.2014.07.024. Epub 2014 Aug 1.
6
The splicing factor U1-70K interacts with the SMN complex and is required for nuclear gem integrity.剪接因子U1-70K与运动神经元存活蛋白复合体相互作用，是核宝石体完整性所必需的。
J Cell Sci. 2014 Sep 15;127(Pt 18):3909-15. doi: 10.1242/jcs.155838. Epub 2014 Jul 22.
7
Spinal muscular atrophy: journeying from bench to bedside.脊髓性肌萎缩症：从实验室到临床的历程
Neurotherapeutics. 2014 Oct;11(4):786-95. doi: 10.1007/s13311-014-0293-y.
8
Autophagy dysregulation in cell culture and animals models of spinal muscular atrophy.脊髓性肌萎缩症细胞培养和动物模型中的自噬失调
Mol Cell Neurosci. 2014 Jul;61:133-40. doi: 10.1016/j.mcn.2014.06.006. Epub 2014 Jun 28.
9
Golgi fragmentation in pmn mice is due to a defective ARF1/TBCE cross-talk that coordinates COPI vesicle formation and tubulin polymerization.嗜中性粒细胞增多症（pmn）小鼠中的高尔基体碎片化是由于ARF1/TBCE相互作用缺陷所致，这种相互作用协调了COP I囊泡的形成和微管蛋白聚合。
Hum Mol Genet. 2014 Nov 15;23(22):5961-75. doi: 10.1093/hmg/ddu320. Epub 2014 Jun 20.
10
Time-resolved quantitative proteomics implicates the core snRNP protein SmB together with SMN in neural trafficking.时间分辨定量蛋白质组学表明核心 snRNP 蛋白 SmB 与 SMN 一起参与神经运输。
J Cell Sci. 2014 Feb 15;127(Pt 4):812-27. doi: 10.1242/jcs.137703. Epub 2013 Dec 19.

α-COP与生存运动神经元蛋白SMN的结合是神经元突起生长所必需的。

α-COP binding to the survival motor neuron protein SMN is required for neuronal process outgrowth.

作者信息

Li Hongxia, Custer Sara K, Gilson Timra, Hao Le Thi, Beattie Christine E, Androphy Elliot J

机构信息

Department of Dermatology, Indiana University School of Medicine, Indianapolis, IN 46202, USA and.

Department of Neuroscience, The Ohio State University, Columbus, OH 43210, USA.

出版信息

Hum Mol Genet. 2015 Dec 20;24(25):7295-307. doi: 10.1093/hmg/ddv428. Epub 2015 Oct 13.

DOI:10.1093/hmg/ddv428
PMID:26464491
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4664168/
Abstract

Spinal muscular atrophy (SMA), a heritable neurodegenerative disease, results from insufficient levels of the survival motor neuron (SMN) protein. α-COP binds to SMN, linking the COPI vesicular transport pathway to SMA. Reduced levels of α-COP restricted development of neuronal processes in NSC-34 cells and primary cortical neurons. Remarkably, heterologous expression of human α-COP restored normal neurite length and morphology in SMN-depleted NSC-34 cells in vitro and zebrafish motor neurons in vivo. We identified single amino acid mutants of α-COP that selectively abrogate SMN binding, retain COPI-mediated Golgi-ER trafficking functionality, but were unable to support neurite outgrowth in cellular and zebrafish models of SMA. Taken together, these demonstrate the functional role of COPI association with the SMN protein in neuronal development.

摘要

脊髓性肌萎缩症（SMA）是一种遗传性神经退行性疾病，由生存运动神经元（SMN）蛋白水平不足所致。α-COP与SMN结合，将COP I囊泡运输途径与SMA联系起来。α-COP水平降低会限制NSC-34细胞和原代皮质神经元中神经突的发育。值得注意的是，人α-COP的异源表达在体外恢复了SMN缺失的NSC-34细胞以及在体内恢复了斑马鱼运动神经元的正常神经突长度和形态。我们鉴定出了α-COP的单氨基酸突变体，这些突变体选择性地消除了与SMN的结合，保留了COP I介导的高尔基体-内质网运输功能，但在SMA的细胞和斑马鱼模型中无法支持神经突生长。综上所述，这些结果证明了COP I与SMN蛋白的结合在神经元发育中的功能作用。