title: Cerebrospinal fluid proteomics and protein biomarkers in frontotemporal lobar degeneration: Current status and future perspectives.
abstract: Frontotemporal lobar degeneration (FTLD) comprises a spectrum of rare neurodegenerative diseases with an estimated prevalence of 15-22 cases per 100,000 persons including the behavioral variant of frontotemporal dementia (bvFTD), progressive non-fluent aphasia (PNFA), semantic dementia (SD), FTD with motor neuron disease (FTD-MND), progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). The pathogenesis of the diseases is still unclear and clinical diagnosis of FTLD is hampered by overlapping symptoms within the FTLD subtypes and with other neurodegenerative diseases such as Alzheimer's disease (AD) and Parkinson's disease (PD). Intracellular protein aggregates in the brain are a major hallmark of FTLD and implicate alterations in protein metabolism or function in the disease's pathogenesis. Cerebrospinal fluid (CSF) which surrounds the brain can be used to study changes in neurodegenerative diseases and to identify disease-related mechanisms or neurochemical biomarkers for diagnosis. In the present review, we will give an overview of the current literature on proteomic studies in CSF of FTLD patients. Reports of targeted and unbiased proteomic approaches are included and the results are discussed in regard of their informative value about disease pathology and the suitability to be used as diagnostic biomarkers. Finally, we will give some future perspectives on CSF proteomics and a list of candidate biomarkers which might be interesting for validation in further studies. This article is part of a Special Issue entitled: Neuroproteomics: Applications in neuroscience and neurology.
authors.affiliation: Department of Neurology, Ulm University Hospital, Oberer Eselsberg 45, 89081 Ulm, Germany. Electronic address: patrick.oeckl@uni-ulm.de.
authors.forename: Patrick
authors.initials: P
authors.lastname: Oeckl
authors.affiliation: Department of Neurology, Ulm University Hospital, Oberer Eselsberg 45, 89081 Ulm, Germany. Electronic address: petra.steinacker@uni-ulm.de.
authors.forename: Petra
authors.initials: P
authors.lastname: Steinacker
authors.affiliation: Department of Neurology, Ulm University Hospital, Oberer Eselsberg 45, 89081 Ulm, Germany. Electronic address: emily.feneberg@uni-ulm.de.
authors.forename: Emily
authors.initials: E
authors.lastname: Feneberg
authors.affiliation: Department of Neurology, Ulm University Hospital, Oberer Eselsberg 45, 89081 Ulm, Germany. Electronic address: markus.otto@uni-ulm.de.
authors.forename: Markus
authors.initials: M
authors.lastname: Otto
mesh_terms: D015415:Biomarkers / Q000134:cerebrospinal fluid; D057174:Frontotemporal Lobar Degeneration / Q000134:cerebrospinal fluid* / Q000175:diagnosis*; D006801:Humans; D009419:Nerve Tissue Proteins / Q000134:cerebrospinal fluid*; D040901:Proteomics / Q000379:methods* / Q000639:trends*
chemical_list: D015415:Biomarkers; D009419:Nerve Tissue Proteins
keywords: Biomarker; CSF; Cerebrospinal fluid; FTLD; Neurodegenerative disease; Proteomic