Deficiency of the zinc finger protein ZPR1 causes neurodegeneration

Doran B; Gherbesi N; Hendricks G; Flavell RA; Davis RJ; Gangwani L

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Deficiency of the zinc finger protein ZPR1 causes neurodegeneration

机译：锌指蛋白ZPR1缺乏导致神经退行性变

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Mutations that cause reduced expression of the full-length Survival Motor Neurons (SMN) protein are a major cause of spinal muscular atrophy (SMA), a disease characterized by degeneration of the a-motor neurons in the anterior horn of the spinal cord. The severity of SMA may be influenced by the actions of modifier genes. One potential modifier gene is represented by ZPR1, which is down-regulated in patients with SMA and encodes a zinc finger protein that interacts with complexes formed by SMN. To test the functional significance of ZPR1 gene down-regulation, we examined a mouse model with targeted ablation of the Zpr1 gene. We report that ZPR1-deficient mice exhibit axonal pathology and neurodegeneration. These data identify ZPR1 deficiency as a contributing factor in neurodegenerative disorders.

机译：导致全长存活运动神经元（SMN）蛋白表达降低的突变是脊髓性肌萎缩症（SMA）的主要原因，该疾病的特征是脊髓前角的a运动神经元变性。 SMA的严重程度可能受到修饰基因作用的影响。 ZPR1代表了一种潜在的修饰基因，该基因在SMA患者中被下调，并编码与SMN形成的复合物相互作用的锌指蛋白。为了测试ZPR1基因下调的功能意义，我们检查了Zpr1基因靶向消融的小鼠模型。我们报告ZPR1缺陷小鼠表现出轴突病理和神经变性。这些数据确定ZPR1缺乏是神经退行性疾病的一个促成因素。

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来源
《Proceedings of the National Academy of Sciences of the United States of America》 |2006年第19期|p. 7471-7475|共5页
作者
Doran B; Gherbesi N; Hendricks G; Flavell RA; Davis RJ; Gangwani L;
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作者单位

Yale Univ, Sch Med, Howard Hughes Med Inst, New Haven, CT 06520 USA;

Yale Univ, Sch Med, Immunobiol Sect, New Haven, CT 06520 USA;

Univ Massachusetts, Sch Med, Howard Hughes Med Inst, Worcester, MA 01605 USA;

Univ Massachusetts, Sch Med, Dept Cell Biol, Program Mol Med, Worcester, MA 01605 USA;

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收录信息美国《科学引文索引》(SCI);美国《生物学医学文摘》(MEDLINE);美国《化学文摘》(CA);
原文格式 PDF
正文语种 eng
中图分类自然科学总论;
关键词
spinal muscular atrophy; SMN; axonopathy; Wallerian degeneration; SPINAL MUSCULAR-ATROPHY; MOTOR-NEURON DEGENERATION; SMN PROTEIN; GENE; MICE; DISEASE; NEUROPATHY; SURVIVAL; LEVEL; MODEL;

机译：脊髓性肌萎缩症;SMN;轴突病;华勒变性;脊髓肌肉萎缩;运动神经元变性;SMN蛋白;基因;小鼠;疾病;神经病;生存;水平;模型;

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机译：锌指蛋白ZFP106缺乏会导致运动和感觉神经变性
2. The zinc finger protein ZPR1 is a potential modifier of spinal muscular atrophy [J] . AhmadS., WangY., ShaikG.M., Human Molecular Genetics . 2012,第12期

机译：锌指蛋白ZPR1是脊髓性肌萎缩症的潜在修饰因子
3. The zinc finger protein ZPR1 is a potential modifier of spinal muscular atrophy [J] . Saif Ahmad2† Yi Wang4†‡ Gouse M. Shaik2¶ Arthur H. Burghes3 and Laxman Gangwani12* Human Molecular Genetics . 2012,第12期

机译：锌指蛋白ZPR1是脊髓性肌萎缩症的潜在修饰因子
4. New architecture for patterning gene expression using zinc finger proteins and small RNAs [C] . Hsia Justin IEEE Conference on Decision and Control;CDC . 2012

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5. New Vistas in Zinc Finger Biochemistry: Examining the Metal-Mediated DNA Recognition of the Neural Zinc Finger Factor/Myelin Transcription Factor Family of Non-Classical Zinc Finger Proteins and Creating Catalytic Moieties from Zinc Finger Scaffolds [D] . Besold, Angelique N. 2014

机译：锌指生物化学的新视野：检查非经典锌指蛋白的神经锌指因子/髓鞘转录因子家族的金属介导的DNA识别，并从锌指支架中产生催化部分
6. Deficiency of the zinc finger protein ZPR1 causes neurodegeneration [O] . Beth Doran, Norberto Gherbesi, Gregory Hendricks, 2006

机译：锌指蛋白ZPR1缺乏导致神经退行性变
7. Deficiency of the zinc finger protein ZPR1 causes neurodegeneration [O] . Doran, Beth, Gherbesi, Norberto, Hendricks, Gregory, 2006

机译：锌指蛋白ZPR1缺乏导致神经退行性变
8. Sumoylation of Heterogeneous Nuclear Ribonucleoproteins, Zinc Finger Proteins, and Nuclear Pore Complex Proteins: A Proteomic Analysis [R] . Li, T. , Evdokimov, E. , Shen, R. , 2004

机译：非均一核核糖核蛋白，锌指蛋白和核孔复合蛋白的构建：蛋白质组学分析

Deficiency of the zinc finger protein ZPR1 causes neurodegeneration

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