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FUS cinating insights into motor neuron degeneration
Author(s) -
Dormann Dorothee
Publication year - 2016
Publication title -
the embo journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 7.484
H-Index - 392
eISSN - 1460-2075
pISSN - 0261-4189
DOI - 10.15252/embj.201694397
Subject(s) - amyotrophic lateral sclerosis , biology , degeneration (medical) , motor neuron , neuroscience , gain of function , loss function , mutant , neuron , neuronal degeneration , function (biology) , motor function , neurodegeneration , mechanism (biology) , disease , microbiology and biotechnology , genetics , phenotype , pathology , gene , physical medicine and rehabilitation , medicine , spinal cord , epistemology , philosophy
Point mutations in FUS cause amyotrophic lateral sclerosis ( ALS ), a devastating neurodegenerative disease—but do they do that by a loss of the protein's normal function, or by endowing it with novel toxic functions, or both? In this issue of The EMBO Journal , Scekic‐Zahirovic et al ([Scekic‐Zahirovic J, 2016]) report that mutant FUS , but not the complete loss of FUS , triggers motor neuron degeneration in mice, arguing for a toxic gain‐of‐function mechanism.