RNA-Binding Protein FUS

Publication Title: 
PloS One

Mutations in the fused in sarcoma/translated in liposarcoma gene (FUS/TLS, FUS) have been identified in sporadic and familial forms of amyotrophic lateral sclerosis (ALS). FUS is an RNA-binding protein that is normally localized in the nucleus, but is mislocalized to the cytoplasm in ALS, and comprises cytoplasmic inclusions in ALS-affected areas. However, it is still unknown whether the neurodegeneration that occurs in ALS is caused by the loss of FUS nuclear function, or by the gain of toxic function due to cytoplasmic FUS aggregation.

Author(s): 
Sasayama, Hiroshi
Shimamura, Mai
Tokuda, Takahiko
Azuma, Yumiko
Yoshida, Tomokatsu
Mizuno, Toshiki
Nakagawa, Masanori
Fujikake, Nobuhiro
Nagai, Yoshitaka
Yamaguchi, Masamitsu
Publication Title: 
Neuromolecular Medicine

Emerging lines of evidence suggest a relationship between amyotrophic lateral sclerosis (ALS) and protein sumoylation. Multiple studies have demonstrated that several of the proteins involved in the pathogenesis of ALS, including superoxide dismutase 1, fused in liposarcoma, and TAR DNA-binding protein 43 (TDP-43), are substrates for sumoylation.

Author(s): 
Foran, Emily
Rosenblum, Lauren
Bogush, Alexey I.
Trotti, Davide
Publication Title: 
PloS One

Genetic mutations of FUS have been linked to many diseases including Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Lobar Degeneration. A primate specific and polymorphic retrotransposon of the SINE-VNTR-Alu (SVA) family is present upstream of the FUS gene. Here we have demonstrated that this retrotransposon can act as a classical transcriptional regulatory domain in the context of a reporter gene construct both in vitro in the human SK-N-AS neuroblastoma cell line and in vivo in a chick embryo model.

Author(s): 
Savage, Abigail L.
Wilm, Thomas P.
Khursheed, Kejhal
Shatunov, Aleksey
Morrison, Karen E.
Shaw, Pamela J.
Shaw, Christopher E.
Smith, Bradley
Breen, Gerome
Al-Chalabi, Ammar
Moss, Diana
Bubb, Vivien J.
Quinn, John P.
Publication Title: 
BMC genomics

BACKGROUND: FUS (TLS) and EWS (EWSR1) belong to the FET-protein family of RNA and DNA binding proteins. FUS and EWS are structurally and functionally related and participate in transcriptional regulation and RNA processing. FUS and EWS are identified in translocation generated cancer fusion proteins and involved in the human neurological diseases amyotrophic lateral sclerosis and fronto-temporal lobar degeneration.

Author(s): 
Luo, Yonglun
Blechingberg, Jenny
Fernandes, Ana Miguel
Li, Shengting
Fryland, Tue
B¯rglum, Anders D.
Bolund, Lars
Nielsen, Anders Lade
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