TAILIEUCHUNG - Báo cáo khoa hoc : TDP-43 and FUS/TLS: cellular functions and implications for neurodegeneration

TDP-43 (transactive response binding protein of 43 kDa) and FUS (fused in sarcoma) comprise the neuropathological protein aggregates of distinct subtypes of the neurodegenerative diseases frontotemporal lobar degen-eration and amyotrophic lateral sclerosis. | IFEBS Journal MINIREVIEW TDP-43 and FUS TLS cellular functions and implications for neurodegeneration Fabienne C. Fiesel and Philipp J. Kahle Department of Neurodegeneration Laboratory of FunctionalNeurogenetics Hertie Institute for ClinicalBrain Research and German Center for Neurodegenerative Diseases Tuebingen Germany Keywords ALS autophagy FTLD FUS HDAC6 RNA RNA metabolism splicing TDP-43 transcription Correspondence F. C. Fieseland P. J. Kahle Department of Neurodegeneration Laboratory of Functional Neurogenetics Hertie Institute for Clinical Brain Research and German Center for Neurodegenerative Diseases Faculty of Medicine University of Tuebingen Otfried Muller Street 27 72076 Tuebingen Germany Fax 49 7071 29 4620 Tel 49 7071 29 81968 49 7071 29 81970 E-mail TDP-43 transactive response binding protein of 43 kDa and FUS fused in sarcoma comprise the neuropathological protein aggregates of distinct subtypes of the neurodegenerative diseases frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Moreover the genes encoding TDP-43 and FUS are linked to these diseases. Both TDP-43 and FUS contain RNA binding motifs and specific targets are being identified. Potential actions of TDP-43 and FUS include transcriptional regulation mRNA processing and micro RNA biogenesis. These activities are probably modulated by interacting proteins in cell type specific manners as well as distinctly within the nucleus and cytosol as both proteins shuttle between these compartments. In this minireview the specific functions of TDP-43 and FUS are described and discussed in the context of how TDP-43 and FUS may contribute to the pathogenesis of frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Received 31 January 2011 revised 23 May 2011 accepted 6 June 2011 doi Abbreviations ALS amyotrophic lateral sclerosis ATM ataxia-telangiectasia mutated Cdk6

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