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ALS-causing mutations in profilin-1 alter its conformational dynamics: A computational approach to explain propensity for aggregation

, USAKottayil I. Varughese AuthorsSearch for Mahmoud Kiaei in:Nature Research journals • PubMed • Google Scholar Search for Meenakshisundaram Balasubramaniam in:Nature Research journals • PubMed • Google ... reviewed and approved in accordance with UAMS conflict of interest policies. Corresponding author Correspondence to Mahmoud Kiaei. Rights and permissions Open Access This article is licensed under a

Nrf2/ARE Signaling Pathway: Key Mediator in Oxidative Stress and Potential Therapeutic Target in ALS

Nrf2 (nuclear erythroid 2-related factor 2) is a basic region leucine-zipper transcription factor which binds to the antioxidant response element (ARE) and thereby regulates the expression of a large battery of genes involved in the cellular antioxidant and anti-inflammatory defence as well as mitochondrial protection. As oxidative stress, inflammation and mitochondrial...

Impaired PGC-1α function in muscle in Huntington's disease

We investigated the role of PPAR γ coactivator 1α (PGC-1α) in muscle dysfunction in Huntington's disease (HD). We observed reduced PGC-1α and target genes expression in muscle of HD transgenic mice. We produced chronic energy deprivation in HD mice by administering the catabolic stressor β-guanidinopropionic acid (GPA), a creatine analogue that reduces ATP levels, activates AMP...

Neuroprotective Effects of the Triterpenoid, CDDO Methyl Amide, a Potent Inducer of Nrf2-Mediated Transcription

The NF-E2-related factor-2 (Nrf2)/antioxidant response element (ARE) signaling pathway regulates phase 2 detoxification genes, including a variety of antioxidative enzymes. We tested neuroprotective effects of the synthetic triterpenoid CDDO-MA, a potent activator of the Nrf2/ARE signaling. CDDO-MA treatment of neuroblastoma SH-SY5Y cells resulted in Nrf2 upregulation and...