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01-09-2009 | Dementias - Original Article

Concentrations of beta-amyloid precursor protein processing products in cerebrospinal fluid of patients with amyotrophic lateral sclerosis and frontotemporal lobar degeneration

Authors: Petra Steinacker, Corinna Hendrich, Anne-Dorte Sperfeld, Sarah Jesse, Stefan Lehnert, Alice Pabst, Christine A. F. von Arnim, Felix M. Mottaghy, Ingo Uttner, Hayrettin Tumani, Albert Ludolph, Markus Otto

Published in: Journal of Neural Transmission | Issue 9/2009

Abstract

Frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) are neurodegenerative disorders with heterogeneous clinical presentation but common neuropathological characteristics and pathophysiological substrates, which led to the view of ALS and FTLD representing two manifestations of a clinicopathological spectrum. For both diseases, changes in metabolism of beta-amyloid precursor protein (APP) are reported. In a pilot study, we analyzed cerebrospinal fluid from patients of the ALS-FTLD spectrum for APP processing products. ALS patients show elevated absolute levels of soluble APP and a shift towards the nonamyloidogenic APP processing pathway in contrast to patients with FTLD or ALS + FTLD. Changes in Aß pattern could be described, allowing separation of patients with pure FTLD from ALS + FTLD. Combination of sAPP and Aß values improves group differentiation. These findings may provide information on pathophysiological processes in the ALS-FTLD disease spectrum and could have impact in neurochemical diagnosis. We propose to expand this study to larger patient groups comprising followed up cases with known neuropathology.

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Title: Concentrations of beta-amyloid precursor protein processing products in cerebrospinal fluid of patients with amyotrophic lateral sclerosis and frontotemporal lobar degeneration
Authors: Petra Steinacker
Corinna Hendrich
Anne-Dorte Sperfeld
Sarah Jesse
Stefan Lehnert
Alice Pabst
Christine A. F. von Arnim
Felix M. Mottaghy
Ingo Uttner
Hayrettin Tumani
Albert Ludolph
Markus Otto
Publication date: 01-09-2009
Publisher: Springer Vienna
Published in: Journal of Neural Transmission / Issue 9/2009
Print ISSN: 0300-9564
Electronic ISSN: 1435-1463
DOI: https://doi.org/10.1007/s00702-009-0271-4

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Concentrations of beta-amyloid precursor protein processing products in cerebrospinal fluid of patients with amyotrophic lateral sclerosis and frontotemporal lobar degeneration

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