In Vitro Seeding Activity of Glycoform-Deficient Prions from Variably Protease-Sensitive Prionopathy and Familial CJD Associated with PrP

Humanized transgenic mice Polymorphism Prion Prion disease Real-time quaking-induced conversion (RT-QuIC) Serial protein misfolding cyclic amplification (sPMCA) Variably protease-sensitive prionopathy (VPSPr)

Journal

Molecular neurobiology
ISSN: 1559-1182
Titre abrégé: Mol Neurobiol
Pays: United States
ID NLM: 8900963

Informations de publication

Date de publication:
Aug 2019
Historique:
received: 12 09 2018
accepted: 17 12 2018
pubmed: 7 1 2019
medline: 18 12 2019
entrez: 7 1 2019
Statut: ppublish

Résumé

Both sporadic variably protease-sensitive prionopathy (VPSPr) and familial Creutzfeldt-Jakob disease linked to the prion protein (PrP) V180I mutation (fCJD

Identifiants

pubmed: 30612334
doi: 10.1007/s12035-018-1459-0
pii: 10.1007/s12035-018-1459-0
pmc: PMC6614145
doi:

Substances chimiques

Prion Proteins 0
Peptide Hydrolases EC 3.4.-

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

5456-5469

Subventions

Organisme : NINDS NIH HHS
ID : R01 NS109532
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS062787
Pays : United States
Organisme : NINDS NIH HHS
ID : R21 NS087588
Pays : United States
Organisme : NIH HHS
ID : NS087588
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS088604
Pays : United States

Commentaires et corrections

Type : ErratumIn

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Auteurs

Zerui Wang (Z)

Department of Neurology, The First Hospital of Jilin University, Changchun, Jilin Province, People's Republic of China.
Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA.

Jue Yuan (J)

Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA.

Pingping Shen (P)

Department of Neurology, The First Hospital of Jilin University, Changchun, Jilin Province, People's Republic of China.

Romany Abskharon (R)

Center for Neurodegenerative Science, Van Andel Research Institute, Grand Rapids, MI, 49503, USA.

Yue Lang (Y)

Department of Neurology, The First Hospital of Jilin University, Changchun, Jilin Province, People's Republic of China.
Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA.

Johnny Dang (J)

Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA.

Alise Adornato (A)

Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA.

Ling Xu (L)

Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA.

Jiafeng Chen (J)

Department of Neurology, The First Hospital of Jilin University, Changchun, Jilin Province, People's Republic of China.

Jiachun Feng (J)

Department of Neurology, The First Hospital of Jilin University, Changchun, Jilin Province, People's Republic of China.

Mohammed Moudjou (M)

Virologie Immunologie Moléculaires, INRA, Jouy-en-Josas, France.

Tetsuyuki Kitamoto (T)

Center for Prion Diseases, Tohoku University Graduate School of Medicine, 2-1 Seiryo-machi, Aoba-ku, Sendai, 980-8575, Japan.

Hyoung-Gon Lee (HG)

Department of Biology, College of Sciences, University of Texas at San Antonio, San Antonio, TX, 78249, USA.

Yong-Sun Kim (YS)

Department of Microbiology, College of Medicine, Hallym University, Chuncheon, Gangwon-do, 24252, Republic of Korea.

Jan Langeveld (J)

Wageningen BioVeterinary Research, Houtribweg 39, Lelystad, the Netherlands.

Brian Appleby (B)

Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA.
National Prion Disease Pathology Surveillance Center, Case Western Reserve University School of Medicine, Cleveland, OH, USA.
Department of Neurology, Case Western Reserve University School of Medicine, Cleveland, OH, USA.

Jiyan Ma (J)

Center for Neurodegenerative Science, Van Andel Research Institute, Grand Rapids, MI, 49503, USA.

Qingzhong Kong (Q)

Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA.
National Prion Disease Pathology Surveillance Center, Case Western Reserve University School of Medicine, Cleveland, OH, USA.
Department of Neurology, Case Western Reserve University School of Medicine, Cleveland, OH, USA.

Robert B Petersen (RB)

Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA. robert.petersen@cmich.edu.
Foundation Sciences, Central Michigan University College of Medicine, Mount Pleasant, MI, USA. robert.petersen@cmich.edu.

Wen-Quan Zou (WQ)

Department of Neurology, The First Hospital of Jilin University, Changchun, Jilin Province, People's Republic of China. wxz6@case.edu.
Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA. wxz6@case.edu.
National Prion Disease Pathology Surveillance Center, Case Western Reserve University School of Medicine, Cleveland, OH, USA. wxz6@case.edu.
Department of Neurology, Case Western Reserve University School of Medicine, Cleveland, OH, USA. wxz6@case.edu.
State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prevention, China Center for Disease Control and Prevention, Beijing, China. wxz6@case.edu.

Li Cui (L)

Department of Neurology, The First Hospital of Jilin University, Changchun, Jilin Province, People's Republic of China. chuili1967@126.com.

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Classifications MeSH