The FUS protein: Physiological functions and a role in amyotrophic lateral sclerosis

• Published in: Molekuliarnaia biologiia Vol. 51; no. 3; p. 387
• Main Authors: Efimova, A D, Ovchinnikov, R K, Roman, A Yu, Maltsev, A V, Grigoriev, V V, Kovrazhkina, E A, Skvortsova, V I
• Format: Journal Article
• Language: Russian
• Published: Russia (Federation) 01.05.2017
• Subjects: Amyotrophic Lateral Sclerosis - genetics; Amyotrophic Lateral Sclerosis - pathology; DNA Repair - genetics; Humans; Motor Neurons - metabolism; Motor Neurons - pathology; Protein Aggregation, Pathological - genetics; RNA Splicing - genetics; RNA-Binding Protein FUS - genetics; RNA-Binding Protein FUS - metabolism; neurodegeneration; FUS; amyotrophic lateral sclerosis; protein aggregation; RNA metabolism
• ISSN: 0026-8984
• DOI: 10.7868/S0026898417020094

Certain forms of amyotrophic lateral sclerosis (ALS) are associated with an altered compartmentalization of FUS and its aggregation in the cytoplasm of motoneurons. FUS is a DNA/RNA-binding protein that is involved in DNA repair and the regulation of transcription, splicing, RNA transport, and local translation. Two theories have been proposed to explain the mechanism of the pathophysiological process in ALS. The theories attribute degeneration of motor neurons to either loss or gain of FUS function. The review describes the main physiological functions of FUS and considers evidence for each of the theories of ALS pathogenesis.