title: Concentrations of beta-amyloid precursor protein processing products in cerebrospinal fluid of patients with amyotrophic lateral sclerosis and frontotemporal lobar degeneration.
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 Abeta pattern could be described, allowing separation of patients with pure FTLD from ALS + FTLD. Combination of sAPP and Abeta 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.
authors: Department of Neurology, University of Ulm, Ulm, Germany.
authors: Petra
authors: P
authors: Steinacker
authors: Corinna
authors: C
authors: Hendrich
authors: Anne-Dorte
authors: AD
authors: Sperfeld
authors: Sarah
authors: S
authors: Jesse
authors: Stefan
authors: S
authors: Lehnert
authors: Alice
authors: A
authors: Pabst
authors: Christine A F
authors: CA
authors: von Arnim
authors: Felix M
authors: FM
authors: Mottaghy
authors: Ingo
authors: I
authors: Uttner
authors: Hayrettin
authors: H
authors: Tumani
authors: Albert
authors: A
authors: Ludolph
authors: Markus
authors: M
authors: Otto
mesh_terms: D000368:Aged; D016229:Amyloid beta-Peptides / Q000134:cerebrospinal fluid; D016564:Amyloid beta-Protein Precursor / Q000134:cerebrospinal fluid*; D000690:Amyotrophic Lateral Sclerosis / Q000134:cerebrospinal fluid*; D019540:Area Under Curve; D003704:Dementia / Q000134:cerebrospinal fluid*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010446:Peptide Fragments / Q000134:cerebrospinal fluid; D012372:ROC Curve
chemical_list: D016229:Amyloid beta-Peptides; D016564:Amyloid beta-Protein Precursor; D010446:Peptide Fragments; C078639:amyloid beta-protein (1-38); C078592:amyloid beta-protein (1-40); C075222:amyloid beta-protein (1-42)