Structural studies of proteins involved in neurodegenerative and muscular diseases 2
参与神经退行性疾病和肌肉疾病的蛋白质的结构研究 2
基本信息
- 批准号:MC_PC_13054
- 负责人:
- 金额:$ 136.94万
- 依托单位:
- 依托单位国家:英国
- 项目类别:Intramural
- 财政年份:2013
- 资助国家:英国
- 起止时间:2013 至 无数据
- 项目状态:已结题
- 来源:
- 关键词:
项目摘要
The accomplishment of the human genome together with the fast progresses made in gene mapping technology have opened for the first time the possibility of approaching hereditary disorders at a molecular level. We approach the study of human diseases by understanding their structure/function relationship. The role of the molecular structure in modern biology and medicine may be directly appreciated if we consider that structure determination of the DNA, the molecule which contains the whole genetic information, unveiled the secret of life. Our laboratory focuses on determining the 3D structure of proteins with specific interest for proteins involved in neurodegenerative diseases such as the Huntington Chorea, Friedreich ataxia, and Fragile X syndrome and muscular pathologies such as different types of cardio-myopathies. Despite the different symptoms, these diseases, all incurable, share the fact that in affected patients, the proteins encoded by the corresponding genes are produced in altered forms, or in smaller quantity down to their complete absence. By determining the shape of the proteins involved we can then understand the role of these mutations on the protein functions and ultimately design new therapeutical strategies. We use a combination of biochemical and biophysical techniques which help us to reconstruct the cellular role of the proteins of interest and characterise their structure/function relationship."
人类基因组的完成以及基因图谱技术的快速进步首次为在分子水平上探讨遗传性疾病打开了可能性。我们通过了解疾病的结构/功能关系来研究人类疾病。如果我们考虑到DNA的结构决定,这个包含全部遗传信息的分子揭示了生命的秘密,那么分子结构在现代生物学和医学中的作用可能会被直接认识到。我们的实验室专注于确定与神经退行性疾病(如亨廷顿舞蹈病、Friedreich共济失调和脆性X综合征)以及肌肉病理(如不同类型的心肌疾病)有关的蛋白质的3D结构。尽管症状不同,但这些都是无法治愈的疾病,都有一个共同的事实,即在受影响的患者中,由相应基因编码的蛋白质会以不同的形式产生,或者数量较少,直到完全缺失。通过确定所涉及的蛋白质的形状,我们可以了解这些突变对蛋白质功能的作用,并最终设计新的治疗策略。我们使用生化和生物物理技术的组合,帮助我们重建感兴趣的蛋白质的细胞角色,并表征它们的结构/功能关系。“
项目成果
期刊论文数量(10)
专著数量(0)
科研奖励数量(0)
会议论文数量(0)
专利数量(0)
Kaleidoscopic protein-protein interactions in the life and death of ataxin-1: new strategies against protein aggregation.
- DOI:10.1016/j.tins.2014.02.003
- 发表时间:2014-04
- 期刊:
- 影响因子:15.9
- 作者:de Chiara C;Pastore A
- 通讯作者:Pastore A
Mutant Exon1 Huntingtin Aggregation is Regulated by T3 Phosphorylation-Induced Structural Changes and Crosstalk between T3 Phosphorylation and Acetylation at K6
- DOI:10.1002/anie.201611750
- 发表时间:2017-05-02
- 期刊:
- 影响因子:16.6
- 作者:Chiki, Anass;DeGuire, Sean M.;Lashuel, Hilal A.
- 通讯作者:Lashuel, Hilal A.
The Molecular Bases of the Dual Regulation of Bacterial Iron Sulfur Cluster Biogenesis by CyaY and IscX.
- DOI:10.3389/fmolb.2017.00097
- 发表时间:2017
- 期刊:
- 影响因子:5
- 作者:Adinolfi S;Puglisi R;Crack JC;Iannuzzi C;Dal Piaz F;Konarev PV;Svergun DI;Martin S;Le Brun NE;Pastore A
- 通讯作者:Pastore A
An optimized strategy to measure protein stability highlights differences between cold and hot unfolded states.
- DOI:10.1038/ncomms15428
- 发表时间:2017-05-18
- 期刊:
- 影响因子:16.6
- 作者:Alfano C;Sanfelice D;Martin SR;Pastore A;Temussi PA
- 通讯作者:Temussi PA
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Annalisa Pastore其他文献
Letter to the Editor: Assignment of the 1H, 13C, and 15N Resonances of Holo Isoform 4 of Lethocerus Indicus Troponin C
- DOI:
10.1023/b:jnmr.0000032558.31512.c8 - 发表时间:
2004-07-01 - 期刊:
- 影响因子:1.900
- 作者:
GianFelice De Nicola;Rolf Biekofsky;Geoff Kelly;Bogos Agianian;Feng Qiu;Belinda Bullard;Annalisa Pastore - 通讯作者:
Annalisa Pastore
Regulating Insect Flight Muscle with Troponin
- DOI:
10.1016/j.bpj.2011.11.1261 - 发表时间:
2012-01-31 - 期刊:
- 影响因子:
- 作者:
Anja R. Katzemich;Gian-Felice De Nicola;Kevin R. Leonard;Annalisa Pastore;Belinda Bullard - 通讯作者:
Belinda Bullard
The C-terminal domain of alpha-spectrin is structurally related to calmodulin.
α-血影蛋白的 C 末端结构域在结构上与钙调蛋白相关。
- DOI:
- 发表时间:
1995 - 期刊:
- 影响因子:0
- 作者:
Gilles Travé;Annalisa Pastore;Marko Hyvönen;M. Saraste - 通讯作者:
M. Saraste
Troponin Isoforms and Stretch-activation of Insect Flight Muscle
- DOI:
10.1016/j.bpj.2008.12.1140 - 发表时间:
2009-02-01 - 期刊:
- 影响因子:
- 作者:
Uros Krzic;Gian De Nicola;Vladimir Rybin;Annalisa Pastore;Kevin Leonard;Wolfgang Linke;Belinda Bullard - 通讯作者:
Belinda Bullard
A survey of the primary structure and the interspecies conservation of I-band titin's elastic elements in vertebrates.
脊椎动物I带肌联弹性元件的一级结构和种间保守性调查。
- DOI:
- 发表时间:
1998 - 期刊:
- 影响因子:3
- 作者:
C. Witt;N. Olivieri;T. Centner;B. Kolmerer;Stefania Millevoi;Jane Morell;D. Labeit;Siegfried Labeit;Harald Jockusch;Annalisa Pastore - 通讯作者:
Annalisa Pastore
Annalisa Pastore的其他文献
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{{ truncateString('Annalisa Pastore', 18)}}的其他基金
Understanding the molecular mechanism of iron sulfur cluster biogenesis
了解铁硫簇生物发生的分子机制
- 批准号:
BB/S001832/1 - 财政年份:2018
- 资助金额:
$ 136.94万 - 项目类别:
Research Grant
The mechanism of stretch activation in muscle: a multidisciplinary approach
肌肉拉伸激活机制:多学科方法
- 批准号:
BB/M006824/1 - 财政年份:2015
- 资助金额:
$ 136.94万 - 项目类别:
Research Grant
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