Jean P Vonsattel
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Explore the profile of Jean P Vonsattel including associated specialties, affiliations and a list of published articles.
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10
Citations
552
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Recent Articles
1.
Pottier C, Kucukali F, Baker M, Batzler A, Jenkins G, van Blitterswijk M, et al.
medRxiv
. 2024 Jul;
PMID: 38978643
Frontotemporal lobar degeneration with neuronal inclusions of the TAR DNA-binding protein 43 (FTLD-TDP) is a fatal neurodegenerative disorder with only a limited number of risk loci identified. We report our...
2.
Lin C, Viswanathan A, Chen T, Mitsumoto H, Vonsattel J, Faust P, et al.
Ann Clin Transl Neurol
. 2022 May;
9(7):988-994.
PMID: 35593123
Objective: Pyramidal signs are common but often under-recognized in multiple system atrophy (MSA). The clinicopathological correlates of pyramidal signs in MSA are not well characterized. The present study aims to...
3.
Gupta D, Vinuela A, Green P, Pitman M, Vonsattel J, Fahn S
Mov Disord Clin Pract
. 2020 Oct;
7(Suppl 3):S93-S95.
PMID: 33015235
No abstract available.
4.
Liou C, Tong M, Vonsattel J, de la Monte S
ASN Neuro
. 2019 May;
11:1759091419839515.
PMID: 31081340
Background: Frontotemporal lobar degeneration (FTLD) is the third most common dementing neurodegenerative disease with nearly 80% having no known etiology. Objective: Growing evidence that neurodegeneration can be linked to dysregulated...
5.
Clark L, Chan R, Cheng R, Liu X, Park N, Parmalee N, et al.
PLoS One
. 2015 May;
10(5):e0125204.
PMID: 25933391
Objective: Variants in GBA are associated with Lewy Body (LB) pathology. We investigated whether variants in other lysosomal storage disorder (LSD) genes also contribute to disease pathogenesis. Methods: We performed...
6.
Cebrian C, Zucca F, Mauri P, Steinbeck J, Studer L, Scherzer C, et al.
Nat Commun
. 2014 Apr;
5:3633.
PMID: 24736453
Subsets of rodent neurons are reported to express major histocompatibility complex class I (MHC-I), but such expression has not been reported in normal adult human neurons. Here we provide evidence...
7.
Todd P, Oh S, Krans A, He F, Sellier C, Frazer M, et al.
Neuron
. 2013 Apr;
78(3):440-55.
PMID: 23602499
Fragile X-associated tremor ataxia syndrome (FXTAS) results from a CGG repeat expansion in the 5' UTR of FMR1. This repeat is thought to elicit toxicity as RNA, yet disease brains...
8.
Chen I, Mitsumoto H, Vonsattel J, Hays A
Muscle Nerve
. 2009 Jan;
39(2):234-8.
PMID: 19127576
Amyotrophic lateral sclerosis (ALS) remains a clinical diagnosis without definable biomarkers. The pathomechanism of motor neuron degeneration in ALS has yet to be elucidated. Here we present a case of...
9.
P301L tauopathy: confocal immunofluorescence study of perinuclear aggregation of the mutated protein
Adamec E, Murrell J, Takao M, Hobbs W, Nixon R, Ghetti B, et al.
J Neurol Sci
. 2002 Jul;
200(1-2):85-93.
PMID: 12127682
The clinical and neuropathological features in the P301L tauopathy have been described in several kindreds. In this study, we present findings in two previously unreported patients, evaluated both genetically, neuropathologically,...
10.
Adamec E, Mohan P, Vonsattel J, Nixon R
Acta Neuropathol
. 2002 Jun;
104(1):92-104.
PMID: 12070670
The calcium-activated protease calpain cleaves a variety of biologically important proteins and serves, therefore, as a key regulator of many cellular functions. Activation of both main isoforms, calpain 1 and...