Jl Holton
New York University
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Publication
Featured researches published by Jl Holton.
Movement Disorders | 2006
Felix Geser; Gregor K. Wenning; Klaus Seppi; Michaela Stampfer‐Kountchev; Christoph Scherfler; Martin Sawires; C. Frick; Jean-Pierre Ndayisaba; Hanno Ulmer; Maria Teresa Pellecchia; Paolo Barone; Ht Kim; J Hooker; N Quinn; Adriana Cardozo; E. Tolosa; Michael Abele; Thomas Klockgether; Karen Østergaard; Erik Dupont; Nicole Schimke; Karla Eggert; Wolfgang H. Oertel; Ruth Djaldetti; Werner Poewe; Richard Dodel; François Tison; Imad Ghorayeb; Pierre Pollak; M Kölensperger
The disease‐specific Unified Multiple System Atrophy Rating Scale (UMSARS) has been developed recently and validated for assessing disease severity in multiple system atrophy (MSA). Here, we aimed at (1) assessing rates of disease progression in MSA and (2) validating UMSARS for sensitivity to change over time. Impairment was assessed at two time points 12 months apart using UMSARS Part I (historical review), UMSARS Part II (motor examination), as well as measures of global disease severity, including UMSARS Part IV, Hoehn and Yahr (HY) Parkinsons disease staging, Schwab England Activities of Daily Living (SE ADL), and a three‐point global Severity Scale (SS3). Fifty patients (male:female ratio, 1:0.9; possible MSA, 16%; probable MSA, 84%; MSA‐parkinsonian, 58%; MSA‐cerebellar, 42%) were assessed twice with an interval of 12.3 months. UMSARS II scores progressed by 57.3% (P < 0.0001) and UMSARS I scores by 35.6% (P < 0.0001) in relation to the respective baseline scores with no differences between motor subtypes, diagnostic categories and gender. Significant inverse correlations between (1) UMSARS I or UMSARS II progression and (2) baseline disability measures (i.e., the respective UMSARS or SS3 scores) and disease duration were found. Furthermore, the increases in HY staging, SE ADL and SS3 correlated significantly with UMSARS I, UMSARS II, and UMSARS IV progression. This report is the first prospective study showing rapid annual UMSARS rates of decline in MSA. Our data contribute to the ongoing validation process of UMSARS, and they facilitate the planning and implementation of future neuroprotective intervention trials.
Annals of the New York Academy of Sciences | 2006
Jorge Ghiso; Ruben Vidal; Agueda Rostagno; Leticia Miravalle; Jl Holton; Simon Mead; Tamas Revesz; Gordon T. Plant; B. Frangione
Abstract: Familial British dementia (FBD) is a disorder characterized by the presence of amyloid deposits in cerebral blood vessels and brain parenchyma coexisting with neurofibrillary tangles in limbic areas. The amyloid subunit (ABri) is a 4 kDa fragment of a 266 amino acid type II single‐spanning transmembrane precursor protein encoded by the BRI gene located on chromosome 13. In FBD patients, a single base substitution at the stop codon of this gene generates a larger 277‐residue precursor (ABriPP‐277). Proteolytic processing by a furin‐like enzyme at the C‐terminus of the elongated precursor generates the 34 amino acid ABri that undergoes rapid aggregation and fibrillization. ABri is structually unrelated to all known amyloids including Aβ, the main component of the amyloid lesions in Alzheimers disease (AD), indicating that cerebral deposition of amyloid molecules other than Aβ can trigger similar neuropathological changes leading to neuronal loss and dementia. These data support the concept that amyloid deposition in the vascular wall and brain parenchyma is of primary importance in the initiation of neurogeneration.
Journal of Biological Chemistry | 2001
Jorge Ghiso; Jl Holton; Leticia Miravalle; Miguel Calero; Tammaryn Lashley; Ruben Vidal; H Houlden; Nicholas W. Wood; Thomas A. Neubert; Agueda Rostagno; Plant G; Tamas Revesz; Blas Frangione
Amyloid | 2001
B. Frangione; Tamas Revesz; Ruben Vidal; Jl Holton; Tammaryn Lashley; H Houlden; Nicholas W. Wood; Agueda Rostagno; Plant G; Jorge Ghiso
NEUROBIOL AGING , 23 (1) S454 - S455. (2002) | 2002
Agueda Rostagno; Laura M. Magnotti; Yasushi Tomidokoro; Blas Frangione; Jorge Ghiso; Tamas Revesz; Tammaryn Lashley; Jl Holton
In: Esiri, MM and Lee, MY and Trojanowski, JQ, (eds.) The neuropathology of dementia. (pp. 330-352). Cambridge University Press: Cambridge. (2004) | 2004
Plant G; Jorge Ghiso; Jl Holton; Blas Frangione; Tamas Revesz
In: Kalimo, H, (ed.) Cerebrovascular Diseases. (pp. 94-102). Wiley-Blackwell (2006) | 2006
Tamas Revesz; Jorge Ghiso; Gordon T. Plant; Tammaryn Lashley; Agueda Rostagno; Blas Frangione; Jl Holton
Archive | 2016
Tamas Revesz; Tammaryn Lashley; Jl Holton
弘前醫學 | 2010
Yasushi Tomidokoro; Akira Tamaoka; Jl Holton; Tammaryn Lashley; Blas Frangione; Tamas Revesz; Agueda Rostagno; Jorge Ghiso
Acta Neuropathologica | 2009
Tamas Revesz; Jl Holton; Tammaryn Lashley; Gordon T. Plant; B. Frangione; Agueda Rostagno; Jorge Ghiso