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lüll Amyotrophic lateral sclerosis, frontotemporal dementia and beyond: the TDP-43 diseases Geser F; Martinez-Lage M; Kwong LK; Lee VM; Trojanowski JQJ Neurol 2009[Aug]; 256 (8): 1205-14Ever since the significance of pathological 43-kDa transactivating responsive sequence DNA-binding protein (TDP-43) for human disease has been recognized in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin positive inclusions (FTLD-U), a number of publications have emerged reporting on this pathology in a variety of neurodegenerative diseases. Given the heterogeneous and, in part, conflicting nature of the recent findings, we here review pathological TDP-43 and its relationship to human disease with a special focus on ALS and FTLD-U. To this end, we propose a classification scheme in which pathological TDP-43 is the major disease defining pathology in one group, or is present in addition to other neurodegenerative hallmark pathologies in a second category. We conclude that the TDP-43 proteinopathies represent a novel class of neurodegenerative disorders akin to alpha-synucleinopathies and tauopathies, with the concept of ALS and FTLD-U to be widened to a broad clinico-pathological multisystem disease, i.e., TDP-43 proteinopathy.|Amyotrophic Lateral Sclerosis/genetics/*metabolism/physiopathology[MESH]|Central Nervous System/*metabolism/pathology/physiopathology[MESH]|DNA-Binding Proteins/chemistry/genetics/*metabolism[MESH]|Dementia/genetics/*metabolism/physiopathology[MESH]|Disease Progression[MESH]|Evolution, Molecular[MESH]|Genetic Predisposition to Disease/genetics[MESH]|Humans[MESH]|Nerve Degeneration/genetics/metabolism/physiopathology[MESH]|Neural Pathways/metabolism/pathology/physiopathology[MESH] |