eprintid: 1433423 rev_number: 29 eprint_status: archive userid: 608 dir: disk0/01/43/34/23 datestamp: 2014-06-30 18:41:17 lastmod: 2021-10-04 01:35:33 status_changed: 2014-06-30 18:41:17 type: article metadata_visibility: show item_issues_count: 0 creators_name: Guerreiro, R creators_name: Brás, J creators_name: Wojtas, A creators_name: Rademakers, R creators_name: Hardy, J creators_name: Graff-Radford, N title: A nonsense mutation in PRNP associated with clinical Alzheimer's disease. ispublished: pub divisions: UCL divisions: B02 divisions: C07 divisions: D07 divisions: F86 keywords: Alzheimer's disease, Exome sequencing, Nonsense mutation, PRNP, Prion note: © 2014 The Authors. Published by Elsevier Inc.This is an open access article under the CC BY license (http:// creativecommons.org/licenses/by/3.0/). abstract: Here, we describe a nonsense haplotype in PRNP associated with clinical Alzheimer's disease. The patient presented an early-onset of cognitive decline with memory loss as the primary cognitive problem. Whole-exome sequencing revealed a nonsense mutation in PRNP (NM_000311, c.C478T; p.Q160*; rs80356711) associated with homozygosity for the V allele at position 129 of the protein, further highlighting how very similar genotypes in PRNP result in strikingly different phenotypes. date: 2014-05-27 official_url: http://dx.doi.org/10.1016/j.neurobiolaging.2014.05.013 vfaculties: VFBRS oa_status: green full_text_type: pub language: eng primo: open primo_central: open_green article_type_text: JOURNAL ARTICLE verified: verified_manual elements_source: PubMed elements_id: 958725 doi: 10.1016/j.neurobiolaging.2014.05.013 pii: S0197-4580(14)00376-5 language_elements: ENG lyricists_name: Hardy, John lyricists_name: Louro Guerreiro, Rita lyricists_name: Smalley, June lyricists_name: Tomas Bras, Jose lyricists_id: JHARD28 lyricists_id: RJLOU51 lyricists_id: JASMA87 lyricists_id: JMTOM86 full_text_status: public publication: Neurobiol Aging volume: 35 number: 11 pagerange: 2656.e13-2656.e16 citation: Guerreiro, R; Brás, J; Wojtas, A; Rademakers, R; Hardy, J; Graff-Radford, N; (2014) A nonsense mutation in PRNP associated with clinical Alzheimer's disease. Neurobiol Aging , 35 (11) 2656.e13-2656.e16. 10.1016/j.neurobiolaging.2014.05.013 <https://doi.org/10.1016/j.neurobiolaging.2014.05.013>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/1433423/1/1-s2.0-S0197458014003765-main.pdf