10.3389/fneur.2018.00407.s004 Tillmann Ruland Tillmann Ruland Jolien Wolbert Jolien Wolbert Michael G. Gottschalk Michael G. Gottschalk Simone König Simone König Andreas Schulte-Mecklenbeck Andreas Schulte-Mecklenbeck Jens Minnerup Jens Minnerup Sven G. Meuth Sven G. Meuth Catharina C. Groß Catharina C. Groß Heinz Wiendl Heinz Wiendl Gerd Meyer zu Hörste Gerd Meyer zu Hörste Table_4_Cerebrospinal Fluid Concentrations of Neuronal Proteins Are Reduced in Primary Angiitis of the Central Nervous System.DOCX Frontiers 2018 PACNS CSF mass spectrometry flow cytometry amyloid-beta A4 protein 2018-06-05 04:36:14 Dataset https://frontiersin.figshare.com/articles/dataset/Table_4_Cerebrospinal_Fluid_Concentrations_of_Neuronal_Proteins_Are_Reduced_in_Primary_Angiitis_of_the_Central_Nervous_System_DOCX/6443345 <p>Primary angiitis of the central nervous system (PACNS) is a rare autoimmune vasculitis limited to the CNS often causing substantial disability. Understanding of this disease is impaired by the lack of available biomaterial. Here, we collected cerebrospinal fluid (CSF) from patients with PACNS and matched controls and performed unbiased proteomics profiling using ion mobility mass spectrometry to identify novel disease mechanisms and candidate biomarkers. We identified 14 candidate proteins, including amyloid-beta A4 protein (APP), with reduced abundance in the CSF of PACNS patients and validated APP by Enzyme-linked Immunosorbent Assay (ELISA) in an extended cohort of patients with PACNS. Subsequent functional annotation surprisingly suggested neuronal pathology rather than immune activation in PACNS. Our study is the first to employ mass spectrometry to local immune reactions in PACNS and it identifies candidates such as APP with pathogenic relevance in PACNS to improve patient care in the future.</p>