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TDP-43/FUS in motor neuron disease: complexity and challenges
(2016-10-01)
Amyotrophic lateral sclerosis (ALS), a common motor neuron disease affecting two per 100,000 people worldwide, encompasses at least five distinct pathological subtypes, including, ALS-SOD1, ALS-C9orf72, ALS-TDP-43, ALS-FUS ...
An Inducible Alpha-Synuclein Expressing Neuronal Cell Line Model for Parkinson’s Disease
(2018-11-08)
Altered expression of α-synuclein is linked to Parkinson’s disease (PD). A major challenge to explore how the increased α-synuclein affect neurotoxicity is the lack of a suitable human neuronal cell model that mimics this ...
New perspectives on oxidized genome damage and repair inhibition by pro-oxidant metals in neurological diseases
(2014-07-17)
The primary cause (s) of neuronal death in most cases of neurodegenerative diseases, including Alzheimer’s and Parkinson’s disease, are still unknown. However, the association of certain etiological factors, eg, oxidative ...
Chromatin-bound oxidized α-synuclein causes strand breaks in neuronal genomes in in vitro models of Parkinson's Disease
(2019-09-01)
Alpha-synuclein (α-Syn) overexpression and misfolding/aggregation in degenerating dopaminergic neurons have long been implicated in Parkinson’s disease (PD). The neurotoxicity of α-Syn is enhanced by iron (Fe) and other ...
Motor neuron disease-associated loss of nuclear TDP-43 is linked to DNA double-strand break repair defects
(2019-03-05)
Genome damage and their defective repair have been etiologically linked to degenerating neurons in many subtypes of amyotrophic lateral sclerosis (ALS) patients; however, the specific mechanisms remain enigmatic. The ...
Chromatin-bound oxidized α-synuclein causes strand breaks in neuronal genomes in in vitro models of Parkinson’s disease
(2017-01-01)
Alpha-synuclein (α-Syn) overexpression and misfolding/aggregation in degenerating dopaminergic neurons have long been implicated in Parkinson’s disease (PD). The neurotoxicity of α-Syn is enhanced by iron (Fe) and other ...
Revisiting metal toxicity in neurodegenerative diseases and stroke: Therapeutic potential.
(2015-01-23)
Excessive accumulation of pro-oxidant metals, observed in affected brain regions, has consistently been implicated as a ontributor to the brain pathology including neurodegenerative diseases and acute injuries such as ...