Structural Determinants of the Prion Protein N-Terminus and Its Adducts with Copper Ions

被引：15
|
作者：
Sanchez-Lopez, Carolina [1 ,9 ]
Rossetti, Giulia [2 ,3 ,4 ,5 ]
Quintanar, Liliana [1 ]
Carloni, Paolo [2 ,3 ,6 ,7 ,8 ]
机构：
[1] Ctr Res & Adv Studies Cinvestav, Dept Chem, Mexico City 07360, DF, Mexico
[2] Forschungszentrum Julich, Inst Neurosci & Med INM 9, Wilhelm Johnen Str, D-52425 Julich, Germany
[3] Forschungszentrum Julich, Inst Adv Simulat IAS 5, Wilhelm Johnen Str, D-52425 Julich, Germany
[4] Forschungszentrum Julich, JSC, D-52428 Julich, Germany
[5] Rhein Westfal TH Aachen, Dept Oncol Hematol & Stem Cell Transplantat, Fac Med, Pauwelsstr 30, D-52074 Aachen, Germany
[6] Rhein Westfal TH Aachen, Dept Phys, D-52078 Aachen, Germany
[7] Rhein Westfal TH Aachen, Dept Neurobiol, D-52078 Aachen, Germany
[8] Forschungszentrum Julich, Inst Neurosci & Med INM 11, D-52428 Julich, Germany
[9] Consejo Nacl Invest Cient & Tecn, Inst Biol Mol Celular Rosario, IBR, Ocampo & Esmeralda, RA-2000 Rosario, Argentina
来源：
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES | 2019年 / 20卷 / 01期
关键词：
N-terminal prion protein; copper binding; prion disease mutations; CELLULAR PRION; OCTAREPEAT DOMAIN; ALPHA-CLEAVAGE; BINDING-SITES; FULL-LENGTH; CONFORMATIONAL POLYMORPHISM; THERMODYNAMIC STABILITY; PATHOGENIC MUTATIONS; BETA-CLEAVAGE; METAL-IONS;
D O I：
10.3390/ijms20010018
中图分类号：
Q5 [生物化学]; Q7 [分子生物学];
学科分类号：
071010 ; 081704 ;
摘要：
The N-terminus of the prion protein is a large intrinsically disordered region encompassing approximately 125 amino acids. In this paper, we review its structural and functional properties, with a particular emphasis on its binding to copper ions. The latter is exploited by the region's conformational flexibility to yield a variety of biological functions. Disease-linked mutations and proteolytic processing of the protein can impact its copper-binding properties, with important structural and functional implications, both in health and disease progression.
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页数：15
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