In:
The EMBO Journal, EMBO, Vol. 35, No. 10 ( 2016-05-17), p. 1077-1097
Abstract:
image Truncation of FUS , leading to cytoplasmic mislocalization, as well as loss of FUS leads to perinatal lethality in mice and alterations in RNA expression and splicing. However, only FUS cytoplasmic mislocalization triggers motor neuron degeneration through motor neuron intrinsic toxicity. Cytoplasmic FUS mislocalization leads to perinatal death and motor neuron degeneration in knockin mice. Complete loss of FUS leads to perinatal death in the absence of motor neuron degeneration. Cytoplasmic FUS mislocalization leads to alterations in gene expression and RNA splicing partially overlapping with complete loss of FUS . Selective rescue of cytoplasmic FUS mislocalization in motor neurons prevents motor neuron degeneration, but not perinatal death.
Type of Medium:
Online Resource
ISSN:
0261-4189
,
1460-2075
DOI:
10.15252/embj.201592559
Language:
English
Publisher:
EMBO
Publication Date:
2016
detail.hit.zdb_id:
1467419-1
detail.hit.zdb_id:
586044-1
SSG:
12
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