Aggregation of ALS-linked FUS mutant sequesters RNA binding proteins and impairs RNA granules formation

•Aggregation of ALS-linked FUS mutant sequesters ALS-associated RNA-binding proteins (FUS wt, hnRNP A1, and hnRNP A2).•Aggregation of ALS-linked FUS mutant sequesters SMN1 in the detergent-insoluble fraction.•Aggregation of ALS-linked FUS mutant reduced the number of speckles in the nucleus.•Overpro...

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Veröffentlicht in:	Biochemical and biophysical research communications 2014-09, Vol.452 (3), p.600-607
Hauptverfasser:	Takanashi, Keisuke, Yamaguchi, Atsushi
Format:	Artikel
Sprache:	eng
Schlagworte:	60 APPLIED LIFE SCIENCES AGGLOMERATION ALS Cell Line CELL NUCLEI Cell Nucleus - metabolism Cell Nucleus - pathology CYTOPLASM Cytoplasm - metabolism Cytoplasm - pathology Cytoplasmic Granules - chemistry Cytoplasmic Granules - metabolism Cytoplasmic Granules - pathology DETERGENTS DNA EQUILIBRIUM FLUORESCENCE FUS/TLS Gene Expression GENES HEK293 Cells Heterogeneous Nuclear Ribonucleoprotein A1 Heterogeneous-Nuclear Ribonucleoprotein Group A-B - chemistry Heterogeneous-Nuclear Ribonucleoprotein Group A-B - genetics Heterogeneous-Nuclear Ribonucleoprotein Group A-B - metabolism hnRNP A/B Humans IN-SITU HYBRIDIZATION MESSENGER-RNA MUTANTS Mutation MUTATIONS Neurons - chemistry Neurons - metabolism Neurons - pathology Protein Aggregates Protein Aggregation, Pathological - metabolism Protein Structure, Tertiary PROTEINS RETENTION RNA, Messenger - chemistry RNA, Messenger - genetics RNA, Messenger - metabolism RNA-Binding Protein FUS - chemistry RNA-Binding Protein FUS - genetics RNA-Binding Protein FUS - metabolism SMN1 STRESSES Survival of Motor Neuron 1 Protein - chemistry Survival of Motor Neuron 1 Protein - genetics Survival of Motor Neuron 1 Protein - metabolism TRANSIENTS
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