The biological function of the cellular prion protein: an update

被引：183

作者：

Wulf, Marie-Angela ^{[1
]}

Senatore, Assunta ^{[1
]}

Aguzzi, Adriano ^{[1
]}

机构：

[1] Univ Zurich, Inst Neuropathol, Ramistr 100, CH-8091 Zurich, Switzerland

来源：

BMC BIOLOGY | 2017年 / 15卷

基金：

欧洲研究理事会;

关键词：

AMYLOID-BETA OLIGOMERS; CEREBELLAR PURKINJE-CELLS; KAINATE-INDUCED SEIZURES; LONG-TERM POTENTIATION; KNOCK-OUT MICE; PRP NULL MICE; SYNAPTIC-TRANSMISSION; NEURITE OUTGROWTH; DEFICIENT MICE; IN-VIVO;

D O I：

10.1186/s12915-017-0375-5

中图分类号：

Q [生物科学];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

The misfolding of the cellular prion protein (PrPC) causes fatal neurodegenerative diseases. Yet PrPC is highly conserved in mammals, suggesting that it exerts beneficial functions preventing its evolutionary elimination. Ablation of PrPC in mice results in well-defined structural and functional alterations in the peripheral nervous system. Many additional phenotypes were ascribed to the lack of PrPC, but some of these were found to arise from genetic artifacts of the underlying mouse models. Here, we revisit the proposed physiological roles of PrPC in the central and peripheral nervous systems and highlight the need for their critical reassessment using new, rigorously controlled animal models.

引用

页数：13

共 149 条

[1] The sheddase ADAM10 is a potent modulator of prion disease [J].

Altmeppen, Hermann C. ;

Prox, Johannes ;

Krasemann, Susanne ;

Puig, Berta ;

Kruszewski, Katharina ;

Dohler, Frank ;

Bernreuther, Christian ;

Hoxha, Ana ;

Linsenmeier, Luise ;

Sikorska, Beata ;

Liberski, Pawel P. ;

Bartsch, Udo ;

Saftig, Paul ;

Glatzel, Markus .

ELIFE, 2015, 4 :1-50

[2] Lack of a-disintegrin-and-metalloproteinase ADAM10 leads to intracellular accumulation and loss of shedding of the cellular prion protein in vivo [J].

Altmeppen, Hermann C. ;

Prox, Johannes ;

Puig, Berta ;

Kluth, Mark A. ;

Bernreuther, Christian ;

Thurm, Dana ;

Jorissen, Ellen ;

Petrowitz, Bettina ;

Bartsch, Udo ;

De Strooper, Bart ;

Saftig, Paul ;

Glatzel, Markus .

MOLECULAR NEURODEGENERATION, 2011, 6

[3] Prion Protein and Its Ligand Stress Inducible Protein 1 Regulate Astrocyte Development [J].

Arantes, Camila ;

Nomiz, Regina ;

Lopes, Marilene H. ;

Hajj, Glaucia N. M. ;

Lima, Flavia R. S. ;

Martins, Vilma R. .

GLIA, 2009, 57 (13) :1439-1449

[4] Synthetic amyloid-β oligomers impair long-term memory independently of cellular prion protein [J].

Balducci, Claudia ;

Beeg, Marten ;

Stravalaci, Matteo ;

Bastone, Antonio ;

Sclip, Alessandra ;

Biasini, Emiliano ;

Tapella, Laura ;

Colombo, Laura ;

Manzoni, Claudia ;

Borsello, Tiziana ;

Chiesa, Roberto ;

Gobbi, Marco ;

Salmona, Mario ;

Forloni, Gianluigi .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2010, 107 (05) :2295-2300

[5] Sialic Acid within the Glycosylphosphatidylinositol Anchor Targets the Cellular Prion Protein to Synapses [J].

Bate, Clive ;

Nolan, William ;

McHale-Owen, Harriet ;

Williams, Alun .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2016, 291 (33) :17093-17101

[6] Lethal recessive myelin toxicity of prion protein lacking its central domain [J].

Baumann, Frank ;

Tolnay, Markus ;

Brabeck, Christine ;

Pahnke, Jens ;

Kloz, Ulrich ;

Niemann, Hartmut H. ;

Heikenwalder, Mathias ;

Ruelicke, Thomas ;

Buerkle, Alexander ;

Aguzzi, Adriano .

EMBO JOURNAL, 2007, 26 (02) :538-547

[7] Aβ induces its own prion protein N-terminal fragment (PrPN1)-mediated neutralization in amorphous aggregates [J].

Beland, Maxime ;

Bedard, Mikael ;

Tremblay, Guillaume ;

Lavigne, Pierre ;

Roucou, Xavier .

NEUROBIOLOGY OF AGING, 2014, 35 (07) :1537-1548

[8] NEARLY UBIQUITOUS TISSUE DISTRIBUTION OF THE SCRAPIE AGENT PRECURSOR PROTEIN [J].

BENDHEIM, PE ;

BROWN, HR ;

RUDELLI, RD ;

SCALA, LJ ;

GOLLER, NL ;

WEN, GY ;

KASCSAK, RJ ;

CASHMAN, NR ;

BOLTON, DC .

NEUROLOGY, 1992, 42 (01) :149-156

[9] Healthy goats naturally devoid of prion protein [J].

Benestad, Sylvie L. ;

Austbo, Lars ;

Tranulis, Michael A. ;

Espenes, Arild ;

Olsaker, Ingrid .

VETERINARY RESEARCH, 2012, 43

[10] Developmental influence of the cellular prion protein on the gene expression profile in mouse hippocampus [J].

Benvegnu, Stefano ;

Roncaglia, Paola ;

Agostini, Federica ;

Casalone, Cristina ;

Corona, Cristiano ;

Gustincich, Stefano ;

Legname, Giuseppe .

PHYSIOLOGICAL GENOMICS, 2011, 43 (12) :711-725

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