Jon Beck
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Explore the profile of Jon Beck including associated specialties, affiliations and a list of published articles.
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24
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1311
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Recent Articles
1.
Katrak S, Pauranik A, Desai S, Mead S, Beck J, Brandner S, et al.
Ann Indian Acad Neurol
. 2019 Nov;
22(4):458-461.
PMID: 31736569
It is now known that the inherited prion disease is caused by over 60 different mutations in the Prion protein (PRNP) gene. Four missense mutations at codons 102, 178, 200...
2.
Keogh M, Wei W, Aryaman J, Walker L, van den Ameele J, Coxhead J, et al.
Nat Commun
. 2018 Oct;
9(1):4257.
PMID: 30323172
Somatic mutations during stem cell division are responsible for several cancers. In principle, a similar process could occur during the intense cell proliferation accompanying human brain development, leading to the...
3.
Ryan N, Nicholas J, Weston P, Liang Y, Lashley T, Guerreiro R, et al.
Lancet Neurol
. 2016 Oct;
15(13):1326-1335.
PMID: 27777022
Background: The causes of phenotypic heterogeneity in familial Alzheimer's disease with autosomal dominant inheritance are not well understood. We aimed to characterise clinical phenotypes and genetic associations with APP and...
4.
Ryan N, Biessels G, Kim L, Nicholas J, Barber P, Walsh P, et al.
Neurobiol Aging
. 2015 Sep;
36(12):3140-3151.
PMID: 26410308
Familial Alzheimer's disease (FAD) treatment trials raise interest in the variable occurrence of cerebral amyloid angiopathy (CAA); an emerging important factor in amyloid-modifying therapy. Previous pathological studies reported particularly severe...
5.
Fratta P, Polke J, Newcombe J, Mizielinska S, Lashley T, Poulter M, et al.
Neurobiol Aging
. 2014 Sep;
36(1):546.e1-7.
PMID: 25179228
An expanded hexanucleotide repeat in the C9orf72 gene is the most common genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia (C9ALS/FTD). Although 0-30 hexanucleotide repeats are present in the...
6.
Owen J, Beck J, Campbell T, Adamson G, Gorham M, Thompson A, et al.
Eur J Hum Genet
. 2014 Apr;
22(12):1351-6.
PMID: 24713662
The inherited prion diseases (IPD) are a group of untreatable neurodegenerative diseases that segregate as autosomal dominant traits. Mutations in the prion protein gene (PRNP) were first found to be...
7.
Hensman Moss D, Poulter M, Beck J, Hehir J, Polke J, Campbell T, et al.
Neurology
. 2013 Dec;
82(4):292-9.
PMID: 24363131
Objective: In many cases where Huntington disease (HD) is suspected, the genetic test for HD is negative: these are known as HD phenocopies. A repeat expansion in the C9orf72 gene...
8.
Lashley T, Rohrer J, Mahoney C, Gordon E, Beck J, Mead S, et al.
Neuropathol Appl Neurobiol
. 2013 Nov;
40(4):502-13.
PMID: 24286341
Aims: Frontotemporal lobar degeneration (FTLD) is a progressive neurodegenerative disease and is the second most common form of young onset dementia after Alzheimer's disease (AD). An autosomal dominant pattern of...
9.
Mead S, Gandhi S, Beck J, Caine D, Gallujipali D, Carswell C, et al.
N Engl J Med
. 2013 Nov;
369(20):1904-14.
PMID: 24224623
Background: Human prion diseases, although variable in clinicopathological phenotype, generally present as neurologic or neuropsychiatric conditions associated with rapid multifocal central nervous system degeneration that is usually dominated by dementia...
10.
Gill O, Spencer Y, Richard-Loendt A, Kelly C, Dabaghian R, Boyes L, et al.
BMJ
. 2013 Oct;
347:f5675.
PMID: 24129059
Objectives: To carry out a further survey of archived appendix samples to understand better the differences between existing estimates of the prevalence of subclinical infection with prions after the bovine...