Soluble beta-amyloid precursor protein is related to disease progression in amyotrophic lateral sclerosis

• Published in: PloS one 2011-08, Vol.6 (8), p.e23600-e23600
• Main Authors: Steinacker, Petra, Fang, Lubin, Kuhle, Jens, Petzold, Axel, Tumani, Hayrettin, Ludolph, Albert C, Otto, Markus, Brettschneider, Johannes
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; Alzheimer's disease; Amyloid beta-protein; Amyloid beta-Protein Precursor - cerebrospinal fluid; Amyloid precursor protein; Amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - cerebrospinal fluid; Amyotrophic Lateral Sclerosis - diagnosis; Apoptosis; Biology; Biomarkers; Biomarkers - cerebrospinal fluid; Cerebrospinal fluid; Clinical trials; Cross-Sectional Studies; Diagnostic tests; Disease control; Disease Progression; Endoplasmic reticulum; Enzyme-linked immunosorbent assay; Female; Follow-Up Studies; Humans; Immunoassay; Intercellular Signaling Peptides and Proteins - cerebrospinal fluid; Male; Medical research; Medicine; Middle Aged; Movement disorders; Neurodegeneration; Neurodegenerative diseases; Neurofilament Proteins - cerebrospinal fluid; Neurofilaments; Neurology; Neurons; Neuroprotection; NMR; Nuclear magnetic resonance; Parkinson Disease - cerebrospinal fluid; Parkinson Disease - pathology; Parkinson's disease; Patients; Precursors; Prognosis; Protein Isoforms - cerebrospinal fluid; Proteins; ROC Curve; Young Adult; β-Amyloid
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0023600

Biomarkers of disease progression in amyotrophic lateral sclerosis (ALS) could support the identification of beneficial drugs in clinical trials. We aimed to test whether soluble fragments of beta-amyloid precursor protein (sAPPα and sAPPß) correlated with clinical subtypes of ALS and were of prognostic value. In a cross-sectional study including patients with ALS (N = 68) with clinical follow-up data over 6 months, Parkinson's disease (PD, N = 20), and age-matched controls (N = 40), cerebrospinal fluid (CSF) levels of sAPPα a, sAPPß and neurofilaments (NfH(SMI35)) were measured by multiplex assay, Progranulin by ELISA. CSF sAPPα and sAPPß levels were lower in ALS with a rapidly-progressive disease course (p = 0.03, and p = 0.02) and with longer disease duration (p = 0.01 and p = 0.01, respectively). CSF NfH(SMI35) was elevated in ALS compared to PD and controls, with highest concentrations found in patients with rapid disease progression (p