Mechanism explains why damaged DNA amass in ALS neurons

A mechanism that leads to the accumulation of damaged DNA in neurons that characterize amyotrophic lateral sclerosis (ALS). A team of neurodegenerative disease researchers recognizes. ALS is a neurodegenerative disease signalized by the selective and progressive death of upper and lower motor neurons.
This leads to ongoing muscle weakness, and death of the patient usually eventuate within two to five years after the onset of symptoms. In approximately 10% of patients, there is a clear family history. Genome damage and defective rebuild have been linked to neurodegeneration in conditions such as ALS. However, the certain mechanisms involved remain unclear.
Investigators at Houston Methodist Hospital (TX, USA) identified defects – caused by evolution in the RNA/DNA-binding protein FUS – in DNA nick ligation and oxidative damage repair in a subset of ALS patients. FUS rapidly appears at sites of DNA damage, which suggests that it is orchestrating the DNA repair response. The function of FUS in the DNA damage response in neurons involves a direct interaction with histone deacetylase 1 (HDAC1).
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